When Vincenzo Bellini arrived in Venice in mid-December 1829 to oversee La Fenice’s production of his 1827 Milan hit Il Pirata, he may still have been smarting from the recent drubbing administered to Zaira, his rather hastily-conceived opera that had been the young composer’s first out-and-out flop. An anonymous handbill had shown up on the streets of Parma immediately following Zaira’s premiere at the new Teatro Ducale: “anyone who finds the musical inspiration of signor Bellini is besought to take it to the box office of signor Bandini, the impresario, and he will be treated courteously.” That must have hurt.
Boom-and-bust being ever the nature of the theatrical beast, Bellini appears to have taken his punishment in stride. There was no particular reason for him to fret; his rise so far had been meteoric and despite youth and inexperience he was in steady demand throughout Italy. His reputation was blossoming. His fees were escalating. His was a charmed life. as of 1829 there could have been no hint that it was also destined to be tragically short.
Born in Catania, Sicily in 1801 to a poor, sprawling, yet tightly knit family, Bellini was raised and educated in music by his grandfather, a local church musician who gave him perhaps more practical experience than solid technique. When Vincenzo came of age in 1819 he was packed off to Naples, where he entered the real Collegio di Musica, a state-run academy. master teacher Nicolò Zingarelli had such confidence in Bellini’s rapidly evolving gifts that he determined that Vincenzo’s graduation opera would be performed in public by Conservatorio students. the resulting Adelson e Salvini—a soapy trifle set in an Irish castle—turned out to be the sleeper hit of 1825 and became a Sunday afternoon staple for the rest of the year. Adelson’s surprise success led to Bellini’s receiving a commission from Naples’ imposing Teatro San Carlo for a full-length opera. Bianca e Fernando was premiered in May 1826. When Bianca followed in Adelson’s footsteps by becoming a hit, Vincenzo Bellini had indubitably arrived, not only as a professional composer but also—thanks in part to his golden-boy good looks—a sought-after presence in posh Neapolitan salons.
Bellini skipped nimbly past the usual gauntlet of provincial theaters, threadbare productions, and semi-starvation that was the usual lot of the neophyte Italian opera composer. instead, he bounded straight to the top. The agent of his rocket-propelled rise was the roughshod, shady, yet solidly reliable impresario Domenico Barbaia who, together with several equally dicey confederates, managed both the San Carlo and Milan’s La Scala. even if the semi-literate Barbaia’s overriding motivation was money— he ran the lobby casinos that subsidized Italian opera productions off and on until about 1820—his taste was impeccable and his nose for talent was keen. the exact details are lacking, but it’s clear enough that Barbaia snapped up the sparkling young Sicilian with a contract for a show scheduled for October 1827 at La Scala. By April Bellini was in Milan, working industriously on his new project while networking and cultivating contacts. the most significant of those was Felice Romani (1788–1865), a Genoese poet and librettist whose portfolio included collaborations with every leading operatic light of the day, including Rossini, Donizetti, Meyerbeer, Pacini, Mercadante, and Vaccai. Between 1827 and 1833, when their partnership ended in quarrels, Romani and Bellini would collaborate on La Straniera, Zaira, I Capuleti e i Montecchi, La Sonnambula, Norma, and Beatrice di Tenda.
And Bellini’s La Scala debut, Il Pirata, which was a resounding success, surely aided by the mega-wattage of vocal effulgence on stage: Henriette Méric-Lalande, Giovanni Battista Rubini, and Antonio Tamburini. Il Pirata was more than a showcase for superstar singers, however. it marked the definite arrival of Bellini’s unique compositional voice, in particular the liquid and fragrantly ornamented bel canto line—a style of singing stressing ease, purity, and evenness of tone production and an agile and precise vocal technique that was to influence so many romantic composers, including Frédéric Chopin. Bellini was fully aware of the magnitude of his accomplishment. In a letter to his uncle he exulted: “my parents and relations can rejoice together; your nephew has had the good fortune to produce such a success with his opera that he does not know how to put it into words.”
In the afterglow of Il Pirata’s starburst, Bellini went back to La Scala for another go at bottling lightning: La Straniera, a barely coherent but attractively creepy gothic tale that inspired Bellini to fashion a brilliantly radical score. Despite its emphasis on austere declamation over lyric numbers, La Straniera delighted Milanese audiences. Then came May 16, the Teatro Ducale in Parma, and Zaira. Romani dawdled at fashioning a workable libretto from Voltaire’s tragedy. Bellini seemed emotionally disconnected from the project, even distracted. the audience included a grumpy contingent with a bone to pick over the chosen libretto. Zaira died, but at least it bequeathed its best parts to posterity. Bellini would mine it extensively for his next opera, I Capuleti e i Montecchi, the happy byproduct of his December 1829 trip to Venice to oversee the production of Il Pirata.
The management of Venice’s Teatro La Fenice had good reason to suspect that the ailing composer Giovanni Pacini would be unable to deliver his setting of Romani’s proposed Giulietta Capellio for the 1829 Carnival season, so they secured a proactive, just-in-case contract with Bellini. that was a smart move. Pacini never showed up and the opera—now a six-week rush job— landed in Bellini’s lap. there was no time for Romani to write a new libretto, so he dusted off his 1825 Giulietta e Romeo for Nicola Vaccai. The equally pressed Bellini ransacked his earlier operas for appropriate material, not only the hapless Zaira but reaching as far back as Adelson e Salvini. It was an exhausting sprint for both of them, exacerbated no doubt by dreadful winter weather that contributed to Bellini’s nasty attack of “sudden bilious gastric inflammatory fever”—an ominous precursor of the dysentery that would kill him five years later.
The enervating trial of its composition notwithstanding, I Capuleti e i Montecchi erased any lingering memory of Zaira’s failure with a triumphant premiere on March 11, capped three days later by the Venetians treating Bellini to a torch-lit parade complete with military band banging out hit tunes from the show. But not all were amused. Hector Berlioz was passing through Florence when I Capuleti was playing at the Pergola theater. He entered the Pergola practically salivating at the prospect of an evening spent in communion with his beloved Shakespeare. He left the Pergola spitting tacks. “Bitter disappointment! no ball at the Capulets’, no Mercutio … no Shakespeare, nothing—a wasted opportunity,” he fulminated in his Memoires, eviscerating “this wretched libretto carved out of Shakespeare’s great play.”
Berlioz was unaware that Romani’s libretto had little if anything to do with Shakespeare’s Romeo and Juliet—a common misconception even today. The most likely source was the 1818 play Giulietta e Romeo by Luigi Scevola, with perhaps some extra material from Nicolò Zingarelli’s 1796 opera, neither directly derived from Shakespeare. Thus I Capuleti e i Montecchi offers its own take on the Romeo and Juliet story, one without those familiar accoutrements such as Mercutio, the balcony scene, or the nurse.
Berlioz had another nit to pick. the female Romeo stuck in his craw. “to write Romeo’s part for a woman—as though there were some law that Juliet’s lover must always appear shorn of his manhood … Moses or Othello discharged in a piping treble would hardly be more incongruous than a female Romeo.” Incongruous, yes; uncommon, no. As of 1830 the trouser role was on its last legs, so to speak. It appears to have originated when women substituted for castrati—singers castrated in boyhood to preserve the soprano or contralto range of their voices—in Baroque operas. that led to the practice of writing male roles explicitly for women, keeping with a general preference for high-voiced heroes, just the opposite of cultural norms today. Romeo in I Capuleti e i Montecchi, written specifically for reigning diva Giuditta Grisi, was to be the last major trouser role in Italian opera. It wasn’t just dead tradition that was driving Bellini; the musical advantages are aptly demonstrated by the ravishing first-act duet (I Capuleti’s equivalent of the balcony scene) in which the complementary ranges of the two singers allow for a close intertwining of vocal lines that could not be achieved with a male Romeo. a related practice of casting females as boys or youths (Cherubino in Mozart’s Le Nozze di Figaro) was to persist; even Berlioz, high dudgeon notwithstanding, got into the trouser-role business with the young apprentice Ascanio in his 1838 Benvenuto Cellini.
As Bellini decompressed from his Capuleti-induced euphoria, he could not have known that he had just crossed an invisible boundary—the one separating his (brief) early period from his (also brief) artistic maturity. I Capuleti e i Montecchi serves as both anthology and capstone of Bellini’s pre-1830 career. Now he was to produce a trio of masterpieces that stand at the very summit of Italian opera. His maturity commenced with a stumble, however, as he and Romani started work on an ill-advised adaptation of Victor Hugo’s controversial play Hernani—which soon proved to be too hot a potato for the Italian censors to handle. Thus chastened, Bellini and Romani turned instead to La Sonnambula, a sweet pastoral drama that won itself a permanent place in the repertory.
On July 23, 1831 Bellini wrote to his friend Alessandro Lamperi: “I have already chosen the subject for my new opera, and it is a tragedy entitled Norma, ossia L’Infanticidio, by Alexandre Soumet.” the tragic story of the druid priestess who goes nobly to the stake for love inspired Bellini to create his juiciest female role and his best-known opera. that said, Norma’s first performance in December 1831 was something of a disaster, but from the second performance onwards Norma was a hit and quickly made its way throughout Europe. it was followed by 1833’s Beatrice di Tenda, a notably less successful venture that ended the Bellini-Romani partnership in a sad fusillade of quarrels.
Paris beckoned. From August 1833, Bellini’s home was the City of Light. The comely young Bellini hobnobbed with Rossini and Chopin as he oversaw productions of Il Pirata and I Capuleti, butted heads with intransigent bureaucrats at the Paris Opéra, and saw his swan song I Puritani through to a wildly successful premiere at the Théâtre-Italien in early 1835. But time was running out. His long-standing intestinal disorder flared up; a liver abscess developed. He died, alone, in suburban Puteaux from amoebic dysentery on September 23, 1835, just five weeks shy of his thirty-fourth birthday. His casket now rests on a marble monument in the Duomo of Catania and bears this line from La Sonnambula: “Ah! I did not think to see you extinguished so soon, o flower.” Bellini’s output may be small, but as in all things, quality trumps quantity. His relatively early I Capuleti e i Montecchi achieves not only a potent blend of dramatic tension and lyrical charm, but also—like the early Shakespeare play upon which it is not based—conjures up a beguiling glimpse of the great things yet to come.
Scott Foglesong has been on the faculty of San Francisco Conservatory since 1978. A pianist and lecturer, he also serves as a contributing writer and “Inside Music” lecturer for San Francisco Symphony.
Note: This essay was published in a 2012 edition of San Francisco Opera Magazine.
"Mefistofeleles is as old as the Bible and Aeschylus. Mefistofele is the serpent in the Garden of Eden; he is the vulture of Prometheus. Mefistofele is the doubt that generates learning, the evil that generates good. Wherever the spirit of negation is to be found, there is Mefistofele. Job has a Mefistofele called Satan; Homer has one called Thersites; Shakespeare has another called Falstaff. Goethe’s original inspiration lies in forming a single type from these three: one who is as hellish as Satan, as grotesque as Thersites, as epi-curean as Falstaff. Mefistofele is the embodiment of the eternal No addressed to the True, the Beautiful and the Good.”
These words from the printed preface to the first edition of Mefistofele tell us more about their author, Arrigo Boito, than about Goethe’s super-devil. Born in Padua in 1842, the son of a Polish countess and a ne’er-do-well miniaturist from the Veneto, Boito was something of an outsider on the Italian scene. His father decamped after two years of marriage, leaving Arrigo’s mother to bring up her two children. Of his family Boito knew only his mother’s relations, but he considered himself wholly Italian with a mission to enlarge his country’s intellectual and artistic horizons and into the vanguard of European culture.
The time was certainly ripe for such an ambition. Under the powers of the Holy Alliance, Italy’s art and literature had tended toward stagnation, and never more palpably than in the decade that fol-lowed the collapse of the 1848 uprising. Even opera, the one musi-cal sphere in which Italy had retained a certain hegemony, was languishing. The post-Rossinian tradition that sustained the inven-tion of Donizetti and Bellini had lost its vitality. Only Verdi remained capable of perpetual self-renewal. Beneath the surface, however, powerful forces were stirring; powers that would break out into the open the moment Italy attained freedom and independence.
The movement known as Scapigliatura (literally, “dishevelment”) took its name from a novel by Carlo Righetti, but its true founder and guide was the writer Giuseppe Rovani, an imposing figure who mentored a host of young artists and litterateurs on aesthetic and moral principles. Their aims were above all iconoclastic. They defied the accepted canons of art and religion and detested the cautious reformers. Thus, the liberal Catholicism of Alessandro Manzoni, the one surviving literary giant of the age, was anathema to them. “Now is the hour of the Antichrist,” the poet Emilio Praga proclaimed. “Christ has died again.”
Returning to Milan in 1863 after two years abroad, Boito threw himself heart and soul into the movement, becoming one of its leading propagandists both in music and literature. As a theatrical critic he attacked the entire apparatus of the established church: “Our generation... cries aloud that every day Catholicism crumbles, that fetishism is in ruins, that a restless search for truth… is rear-ing its head. It cries continually that God has putrefied, that man has made himself divine, that the Man–God no longer exists, that Genius alone is the Son of God... that the Holy Spirit is no longer amongst us, that there is no Easter, no Virgin, and no Resurrection...” and so on. His most characteristic product of those years was a bizarre poem, II Re Orso (King Bear) about a monarch haunted by a mysterious voice that bids him beware of the “worm’s bite”; and of course it is the worm who wins in the end, devouring the king’s dead body. The general layout, with its recur-rent motifs and wealth of verbal assonance, gives the sense of a symphonic poem in words, and not by chance.
For Boito, music remained the supreme art—"the queen of all the arts; more than a queen: a goddess." He preached the regeneration of the long-lost Italian instrumental tradition chiefly through the study of the great German classics, whose authors he discoursed upon in bursts of high-flown imagery, not always comprehensible. “Haydn proceeds from Bach like the flowering cytisus from the terrible rock.” In Schumann’s music it is not Schumann who sings, “it is a Sybil, a grace, a zvango [a word for which you will search in vain in an Italian dictionary], a god. Every time the critic’s pen encounters the name of Beethoven it should pause before it, like a pilgrim before a cross.” The comparison of a Mozart slow movement with one by Mendelssohn prompted him to a notorious blaze of rhetoric:
The Sublime is simpler than the beautiful. The Beautiful can become incarnate in every kind of form from the bizarre to the multiple and disparate: for the Sublime only one form will suffice: the grand form, the form that is divine, universal, eternal—the spherical form. The horizon is sublime, the sea is sublime, the sun is sublime. Shakespeare is spherical, Dante is spherical, Beethoven is spherical; the sun is simpler than a carnation, the sea simpler than the brook, Mendelssohn’s Adagio is spherical and simpler than Mozart’s Andante.”
For Boito, however, the ultimate goal always had to be opera. “Let us practice the symphony and the quartet,” he declared, “so as to be able to tackle the lyric drama.” Not the kind of opera to which Italians had long been accustomed. To the post-Rossinian tradition with its abundance of fixed patterns such as the aria, the duet, the pezzo concertato, he opposed a firm Mephistophelian “No!” These were mere formulae—a term that by its etymological derivation revealed its essentially trivializing nature. What was wanted was form in the grandest sense. But how was it to be achieved? Evidently not with the help of Wagnerian methods.
“Wagner,” Boito admitted, “destroyed the operatic formula, Wagner promised to enlarge the bounds of rhythm and melody, Wagner in his triple role as poet, composer, and aesthetic philosopher seemed to be the man born and predestined to accomplish the mission of renewal… It would be unkind of us not to recognize in his music a powerful instinct, a vigorous muscular frame, but his dramas are inept and ridiculous in the face of the task they were called upon to undertake.”
Clearly it was up to Boito himself to set an example for others to follow; in due course the opportunity presented itself. Among the themes that occupied Boito throughout his life was the polarity of good and evil, and nowhere did he see this problem more compre-hensively worked out than in the monumental poetic drama of Goethe. The notion of Faust as an operatic subject seems to have occurred to him during his conservatory days in the 1850s; by 1867 its realization seemed a possibility. The venue was to be Milan’s La Scala, where the musical director was Boito’s old teacher Alberto Mazzucato. He could also count on the support of Filippo Filippi, the leading critic of the time and himself a member of the Scapigliatura, and that of Giulio Ricordi, a close personal friend and the effective head of his ailing father’s publishing firm.
It was not Boito’s first venture into the operatic world. In 1865 he had already written a libretto for his friend Franco Faccio’s Amleto. But Mefistofele, as it would be called, was to be very different: a large-scale music drama exploring the German poet’s thought in all its ramifications without any concessions to current practice. Its only conventional feature was to be Mefistofele’s aria “Son lo spirito che nega,” cast in the form of French couplets in the manner of Meyerbeer (a composer, incidentally, much admired by Boito). Even this would contain its own condemna-tion: a refrain consisting of loud whistles, the traditional [Euro-pean] sign of audience disapproval. At the premiere, alas, this turned out to be more than a metaphor.
The first performance took place on March 5, 1868, and Boito himself unwisely assumed the direction. Not everything was disliked. There was some applause for the Prologue in Heaven and for the quartet in the garden scene, while the Classical Sabbath was heard in respectful silence. For the rest of the score, whistles and boos were the order of the day. In view of the opera’s immense length (the show ended at half-past one in the morning) it was decided to revive it in two parts, to be given on successive evenings. But in this form, the opera fared no better. The singers were hopelessly demoralized, and amid shouts of “Basta!” the cur-tain fell on March 8 on the original Mefistofele, never to rise again.
The critics were no less hostile. Some accused Boito fatuously of having imitated Wagner, whose name was then on everyone’s lips, though few had heard a note of his music. Typical of the general consensus was a notice that appeared in L’Emporia Pittoresco: “The music of Mefistofele could not please.... There is no passion, and the monotony of the recitative ends by boring. Boito should realize that he has given a far more convincing proof of his talent for literature, and his failure may turn out to be a blessing if it induces him to cultivate that field and no other.” Nor was the verdict of Boito’s friend Giulio Ricordi very different, though expressed in more diplomatic terms. “Boito has written an opera with many virtues and not a few defects. The question is: are these defects due to inexperience as regards the stage and matters theatrical? In that case, so much the better; we shall note a steady progress from one opera to the next, and in due course I shall hope to number Boito among the great composers. If, however, these faults are the result of a preconceived theory, of an unshakable artistic conviction, then I must say with all the frankness which informs my warm and deeply felt friendship for Boito: you may be a poet and a distinguished man of letters, but you will never be a composer for the musical stage.”
Unfortunately, we cannot judge the matter for ourselves since no holograph of the 1868 version of Mefistofele exists, nor was it ever published in vocal score. When he came to revise it seven years later, Boito tore out and destroyed the pages that he wished to replace. No trace remains of a dialogue between Faust and his disciple Wagner on the real and the ideal, an encounter with Lilith on the Brocken followed by a “black” Miserere, and an extended scene set in the throne room of an emperor to whom Mefistofele presents himself as a new jester. Two pieces were added: the duettino “Lontano, lontano, lontano,” lifted from Boito’s unfinished Era e Leandro and Margherita’s prison aria, “Spunta l’aurora pallida.” The effect of the revision was to reduce the opera to manageable proportions and a somewhat more traditional layout. The five acts became four; and Faust was transformed from a baritone into a tenor.
The premiere of the new Mefistofele took place on August 5th, 1875, at the Teatro Comunale, Bologna, which prided itself on its forward-looking policy (in 1871 it had witnessed the first Italian performance of a Wagner opera, namely Lohengrin). The result, if not an uncontested triumph, was enough of a success to permit a circulation among the major theaters of the peninsula. For a revival in Venice the following year, Boito made a further modification, after which the opera entered the international repertoire. Nowhere was it better received than in England, where it would feature in several Covent Garden seasons towards the end of the century. Reviewing the revival of 1889, George Bernard Shaw wrote: “Boito’s version of the Faust story seems almost as popular as Gounod’s”—surely an overstatement, considering that the French work was given at Covent Gar-den every year from 1863 until 1911— ”though Gounod’s is a true musical creation whereas Boito has only adapted the existing resources of orchestration and harmony very ably to his libretto. The whole work is a curious example of what can be done in opera by an accomplished literary man without original musical gifts, but with ten times the taste and culture of a musician of only ordinary extraordinariness.”
A shrewd judgment, this, like so many of Shaw’s on Italian opera. It is true that Boito never developed a musical style that is instantly recognizable as his own. That of Mefistofele is distinctly eclectic. More than one critic noted the debt of Faust’s solo “Dai campi, dai prati” to the slow movement of Beethoven’s Kreutzer Sonata; anyone familiar with the love duet in Franco Faccio’s I Profughi Fiamminghi would have found a clear precedent for Margherita’s “Cavalliero illustre a saggio”; and the main theme of the duet between Faust and Elena belongs to the family of Verdi’s “Dunque l’onta di tutti sol una” in Un Ballo in Maschera.
The years that followed the fiasco of 1868 had taught the com-poser some valuable lessons. He had descended from the clouds of Scapigliatura theorizing to become a practical man of the theater. He wrote several libretti for other composers to use, among them La Gioconda, Ponchielli’s most successful stage work and arguably the only Italian “grand opera” that can stand comparison with Verdi’s Aida. He even came to terms with Wagner, making translations of Rienzi, Das Liebesmahl der Apostolen, and eventually Tristan und Isolde. But his atti-tude to the Master of Bayreuth remained ambivalent. He was stirred by the music, but repelled by the dramaturgy. He compared Die Walküre to a stopping train that takes ages to reach its destination.
By 1870 the Scapigliatura had begun to disintegrate. Many of Boito’s former associates had espoused realism in art and the left wing in politics; he himself held proudly aloof from both. As a writer he inclined towards the subtly allusive metaphor, the pregnant phrase, drawing upon a vocabulary that ranged from medieval times to his own day but always supremely adaptable to musical thought. The chief fruits of his literary labors were gar-nered by the aged Verdi in Otello and, more especially, Falstaff. And indeed Boito was later to claim as his chief glory the fact that he had “made the bronze colossus resound twice.” Meanwhile, as a musician, he found himself increasingly isolated among his own countrymen. He had no sympathy with the “young school” headed by Puccini and Mascagni. He wrestled continually with his most ambitious project, a huge opera on the subject of Nero and his persecution of the Christians. Like Gustav Holst, he suffered bouts of nervous agraphia, during which he was unable to hold a pen. By the time Boito died in 1918, only four of the five acts had been set and not even they were fully scored; six years were to pass before Toscanini with the aid of the composer Vincenzo Tom-masini was able to perform a presentable version of the torso. In Boito, the intellectual had for a long time stifled the creator.
Mefistofele remains his sole musical monument. It is, as Shaw observed, essentially the work of a literary man. But, if not a mas-terpiece of musical organization, several of its ideas are striking and imaginative. There are no lapses of taste; there is never a note or an instrument too many. The chorus “Ave Signor degli angeli,” with its suggestion of perpetual ascent, is unique in opera; nor will you find a more perfect miniature than the duet “Lontano, lon-tano, lontano.” Nowhere is the attention allowed to wander. Lacking both forebears and descendants, Mefistofele is a work that we should be the poorer without.
Professor Stephen Westaby, a British heart surgeon at John Radcliffe Hospital. Supplied
by Stephen Westaby
"We shall never surrender."
Winston Churchill, during the Battle of Britain, 1940
Monday 15 February 1999, 3.45am. No one calls with good news at night. I'd been in Australia for just thirteen hours after a twenty-hour flight. In pitch darkness I scrambled across my hotel bed and knocked the receiver to the floor. The call was lost. I swiftly slipped back into sleep, courtesy of melatonin tablets and the bottle of Merlot I'd drunk at dinner. Ten minutes later the phone rang again. This time I fielded it successfully, but I was irritated.
"Westaby? This is Archer. Where are you?"
Kirsty Collier with her mum Becky before her operation. After having a third of her heart removed in pioneering surgery Kirsty astounded medical experts by making a ''miracle'' recovery. SWNS.com
"Nick, you know I'm damned well in Australia. It's the middle of the f***ing night – what's the problem?"
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I didn't want to hear the answer.
"Steve, I'm sorry, but we need you to come back. We have a sick baby with ALCAPA. The parents know you and want you to do the surgery."
Oh joy. "When?"
"As soon as possible."
Kirsty Collier two months after her operation in February 1999. Kirsty had a third of her heart removed when she was just 4 months old. SWNS.com
I really had no other choice.
Kirsty was a beautiful six-month-old baby girl in whom fate had installed a lethal self-destruct mechanism, a miserable detail that seemed destined to end her life before her first birthday. ALCAPA is an abbreviation for Anomalous Left Coronary Artery from the Pulmonary Artery, an isolated and exceptionally rare congenital anomaly in the overall complexity of human anatomy.
Simply put, it's bad wiring. Both coronary arteries should rise out of the aorta and supply the heart muscle with well-oxygenated blood under high pressure. They should never be attached to the pulmonary artery, as this has both low pressure and poor oxygen content. Early survival with ALCAPA therefore depends on the development of new "collateral" blood vessels between the normal right coronary artery and the misplaced left coronary artery. But these eventually are insufficient to sustain blood flow to the main pumping chamber.
Muscle cells deprived of oxygen die and are replaced by scar tissue, leaving baby to suffer what are in effect repeated, painful heart attacks. Scar tissue stretches, causing the left ventricle to dilate, then the heart progressively fails and the lungs become congested with blood, leading to breathlessness and exhaustion. Even during feeding.
Kirsty as a 10-year-old with her mother Becky Collier. SWNS.com
But because ALCAPA is exceedingly rare, the diagnosis is seldom made until the infant is terminally ill. Fortunately, her parents were intelligent, had recognised that there was a serious problem and were persistent in finding help for her.
Kirsty's story was particularly harrowing. Her mother Becky already had a three-year-old son and was an experienced and responsible mum. All antenatal checks and ultra-sound scans had appeared normal. Kirsty was born on August 21, 1998, by elective Caesarean section with a spinal anaesthetic and at first everything seemed normal. But not for long.
In the womb, the pressures and oxygen content of the aorta and pulmonary artery are the same, so Kirsty's tiny heart was safe. After birth, the circulations to the body and newly expanded lungs separate, and both pressure and oxygen content in the pulmonary artery fall. So, in cases of ALCAPA, both blood flow and oxygen content in that critically important left coronary artery fall precipitously, too.
Kirsty was grunting even during the first attempt to breastfeed in the hospital, and Becky noticed beads of sweat trickling from the bridge of her baby's nose. The effort of feeding repeatedly made her fractious and distressed.
Kirsty with her parents before her school prom. Kirsty made a full recovery and aside from a feint scar on her chest has little to show for the surgery. Supplied
This was distinctly different from how her son had been, so Becky asked for a paediatrician to review Kirsty. She was told that there was absolutely nothing to worry about but the truth was that no one had bothered to find out what was wrong.At this stage, Becky had little choice but to take her irritable but precious little bundle home.
Within weeks Becky was certain that something was seriously amiss, because during every feed there was sweating and vomiting. Kirsty struggled for breath, clenched her little fists and screamed until she was puce in the face. Together they made many visits to the GP, sometimes as many as three times a week, but they always received the same non-committal reassurance – tense, unpleasant encounters, as Becky was deemed neurotic and unable to cope.
Despite Kirsty's rapid breathing she had no temperature. Chest infections were ruled out, and her belly was soft, with no signs of stomach or intestinal blockage. All of the common paediatric problems were excluded. Becky became more and more anxious. Kirsty wasn't gaining weight. She had a pasty, washed-out look and a cough like a dog's bark.
In reality this baby was suffering repeated small heart attacks with excruciating chest pain that she could neither communicate nor understand. The human body can be outlandishly cruel.
FRAGILE LIVES Illustrations labeling and depicting elements of the heart and cardiovascular systems. Heart Surgery Prodger, Isabel
Eventually, after a meltdown in the GP's surgery, Becky insisted that Kirsty should be referred to the local hospital. Twice she had chest X-rays, only to be diagnosed with bronchiolitis – inflamed breathing tubes – on both occasions. Then one day during her afternoon nap Kirsty turned a terrible slate-grey colour. She was barely rousable and limp. Becky snatched her up and rushed to the surgery. But by the time they presented themselves at the receptionist the baby was awake and pink again.
Becky was told to stop fussing and that there were children to be seen who were actually sick. On this occasion mother and child were acrimoniously dispatched, once more with a prescription for antibiotics. Kirsty's enormous heart remained undetected.
This time, Becky drove directly to the accident and emergency department of her small local hospital. They were seen by a sympathetic female doctor who had children of her own. Recognising that a mother's instinct was usually correct, she referred them on to a larger city hospital for review by the on-call paediatrician.
It was a bitterly cold, frosty night and they were left sitting in an unheated hospital corridor for several hours. Becky frantically struggled to keep Kirsty warm but she became progressively more limp and grey. Eventually, late into the night, they were seen. The first junior doctor suggested bronchiolitis and intended to dismiss them without investigation.
Beckyrefused to leave without a chest X-ray. She was told off for her unreasonable attitude – how thoughtless to inconvenience the hard-pressed duty radiographer at that time of night. So the sad pair were dispatched unescorted down poorly lit corridors and icy outside walkways to find their own way to the X-ray department. It was well past midnight when they returned, clutching the tell-tale picture, which Becky presented to a nurse. They were parked once more.
Another thirty minutes passed, then a dramatic shift in attitude from the hospital staff. Now there were hushed voices, grave expressions, and nurses bringing drips and drugs. The previously mean, now embarrassed nurse took Becky aside to explain that Kirsty was being transferred to the specialist children's heart unit in Oxford. By ambulance this time.
So what did the X-ray show to trigger this frenzy of activity? Kirsty had a massive heart. Her problem was immediately obvious on the X-ray film. When the staff were pressed about her previous X-rays at the same hospital, Becky was told that the heart shadow had been misinterpreted as fluid. Some mistake!
In Oxford, the paediatric cardiology registrar met the ambulance and took them directly to a ward packed with children with serious heart problems and beeping monitors – a hive of activity in the depths of night.
FRAGILE LIVES Illustrations labeling and depicting elements of the heart and cardiovascular systems. Heart Surgery Prodger, Isabel
Nick Archer arrived at 3am. An ECG and blood tests were done quickly, then the echo machine was brought in to image Kirsty's heart chambers. First it seemed like good news. All four chambers were there, with no holes between them. But worryingly the left atrium and ventricle were both enlarged, the ventricle dramatically so. This explained the heart failure and accounted for the striking chest X-ray.
In little more than an hour, the cardiology team established that Kirsty had severe heart failure from multiple heart attacks. Parts of the left ventricular wall now consisted of thin scar tissue interspersed with poorly contracting muscle, a rare condition in an infant but one that provided the likely diagnosis. One further test was needed. A cardiac catheter would confirm the diagnosis but would require a general anaesthetic, so she'd have to be in much better condition before proceeding.
It was a full five weeks before Becky's baby was considered fit enough for the cardiac catheter. The evening before the operation, the anaesthetist came round to talk. Normally a jolly and optimistic character, on this occasion he didn't have much to smile about. That night, Kirsty was christened in her cot by the hospital chaplain, the doctors, nurses and other families gathering around the bed to support them.
That morning, Becky dressed her baby in her best pyjamas and tied a bow in her hair for her trip to the catheterisation laboratory. Ironically it was Valentine's Day. As Becky put it to me later, "A girl has to look her best, even when she's poorly.":
Once I was on the plane home I started to sketch the anatomy of Kirsty's aorta, pulmonary artery and abnormal left coronary artery. I knew that the current techniques in operations for ALCAPA had limitations and a substantial failure rate, so I used my time during the flight to work out an alternative. By the time we were cruising high above Java, I'd designed my new operation.
I called my colleague Takahiro Katsumata when I got back to Oxford and asked him to bring Kirsty's parents to my office with a consent form. The cardiac catheter had shown precisely what Archer had suspected. Kirsty needed surgery as soon as possible.
Becky looked tired and drawn when I saw her.
I don't do transmitted anxiety if I can help it. But it's much more difficult for my anaesthetist colleagues who have to deal with the agonising separation when the patient is given over to them.
FRAGILE LIVES Illustrations labeling and depicting elements of the heart and cardiovascular systems. Heart Surgery Prodger, Isabel
I described my planned operation to the team and explained why I felt it would be an improvement on existing techniques. The new left coronary artery would be constructed with a flap of aortic wall that would sit below a corresponding pulmonary flap to make a tube, the latter containing the misplaced origin of the left coronary artery at its apex. The product would be a new coronary artery delivering high-pressure, well-oxygenated blood directly from the aorta, where it should have come from in the first place. Blood fully saturated with oxygen would then supply the failing heart muscle and prevent further heart attacks. Katsumata was intrigued and excited by my proposed approach, so much so that he rushed off to call the hospital's film crew.
The risks of the operation were substantial. Becky's shaky hand signed the consent form and I walked back with them to the children's ward. When we reached the cot, Kirsty's heart failure was worse than I imagined. She was emaciated, her heaving ribs and rapid breathing a consequence of her congested lungs, and her abdomen swollen with fluid. Without immediate surgery she'd be dead within days. Although a voice in my head screamed, "Oh shit," my mouth correctly said, "I'll go to theatre now."
The anaesthetist knew the score, having already anaesthetised Kirsty for the cardiac catheterisation, and some of the monitoring lines were still in place.
"Do you really think you can get this baby through?" was his opening line.
I didn't reply, bidding a cheery "Good morning" to the nurses and perfusion team in the operating theatre, then went straight to the coffee room. I wanted to avoid seeing Becky leave her baby with strangers, always an excruciating event.
When I returned, Kirsty was already on the operating table, covered in green drapes held in place with an adherent plastic drape. All that was visible were her bony little chest and swollen abdomen. Heart surgery needs to be an impersonal, technical exercise.
I joined Katsumata and my six-foot, six-inch Australian colleague Matthew at the scrub sink. While we scrubbed in silence, the film camera was carefully positioned next to the operating lights. There was a palpable buzz of excitement. We were about to do something novel, esoteric and risky.
There was no bleeding as I drew the scalpel blade along the skin over Kirsty's breast bone. In shock, her skin capillaries had shut down to divert blood to vital organs. Next, the electrocauter cut through the thin layer of fat onto the bone, producing its characteristic buzzing noise accompanied by a whiff of burning as the current cauterised the oozing blood vessels, although this time there were few of these. Then the electric saw cut through the length of her sternum, exposing bright red bone marrow.
We used a small metal retractor to crank open her tiny chest, bending and stretching the joints between the ribs and the spinal column. In babies, the fleshy thymus gland lies between the sternum and the fibrous sac around the heart, but by now it had done its work producing antibodies for the foetus so we removed it.
The electrocauter continued its messy but vital work, cutting through the fibrous pericardial sac to expose the heart, straw-coloured fluid pouring out and being drawn away by the sucker. Meanwhile the other members of the team worked on silently. The anaesthetist gave heparin to stop Kirsty's blood from clotting in the heart–lung machine, the perfusion team set up the complex array of tubing, pumps and oxygenating equipment to keep Kirsty's body alive when her heart was stopped, and the scrub nurse concentrated on having the correct surgical instruments ready to slap into my palm. I rarely had to ask for anything.
As we pulled up the edges of the pericardial membrane to display the heart, Katsumata – in a moment of wry Japanese humour in times of stress – involuntarily uttered what now had become our surgical catchphrase, "Oh shit, don't mention the war." Back from his first cigarette, the anaesthetist popped his head over the drapes in response to Katsumata's comment. Others could see it all on the video screen.
What should have been a walnut-sized heart was revealed to be the size of a lemon. The enlarged right coronary artery was obvious, its many inflated branches crossing over towards the left ventricle. While the right side of the heart pumped vigorously against raised pressure in the lungs, the left ventricle was hugely dilated and barely moved.
Batches of newly necrotic muscle merged with areas of white, fibrous scar tissue, the result of the many small, painful heart attacks endured by Kirsty during her first six months of life. But Kirsty had survived to this point and it was our job to keep it that way.
Having exposed this heart I began to question the wisdom of attempting such a complex operation having come straight from a day-long flight.
But urgent heart transplants were virtually impossible in babies; this replumbing of her heart's blood supply was her only chance of life.
Tiny pipes were inserted to connect her to the heart–lung machine, and I then gave the signal to continue. The perfusion technician turned on the roller pump and Kirsty's heart gradually emptied. At this point, technology had taken over, diverting blood away from her lungs and into the synthetic oxygenator.
With her empty heart still beating I cut through the pulmonary artery above the origin of the anomalous coronary, which sat there like the pearl in an oyster. Now we had to connect it without tension to the high-pressure aorta that lay an inch away. The conventional method was simply to trying to stretch and re-implant the origin of the vessel into the side of the aorta. But this could result in thrombosis and blockage, so I pressed on with my new technique.
This delicate exercise could only be achieved by clamping the aorta and temporarily stopping all blood flow to the heart. We'd protect the muscle by infusing cardioplegia fluid directly into both coronary arteries, flushing all the blood out and collapsing the ventricle like a punctured football. This induced state of inactivity, common in heart surgery, is reversed simply by removing the clamp on the aorta, which allows blood from the heart–lung machine to flow back into the coronary arteries.
For the reconstruction of this tiny vessel, the stitching had to be precise, accurate and watertight. The procedure went well. Just thirty minutes after the heart was stopped, the conjoined flaps restored Kirsty's coronary artery anatomy to what it should have been.
As the clamp was removed, bright red oxygenated blood – rather than de-oxygenated blue blood – flooded the left ventricular muscle. Her heart changed from a pale pink colour to deep purple, then became almost black in parts. Before re-constructing the pulmonary artery we checked that there was no bleeding from the lines of stitching behind it. Soon the electro-cardiogram showed uncoordinated electrical activity, and the heart stiffened with renewed muscle tone.
Unusually for a child, her reperfused heart kept writhing and squirming in ventricular fibrillation. We used an electric shock directly through the muscle to restore normal rhythm. Ten volts – zap! The heart defibrillated and stopped wriggling. It was now motionless but we expected a normal rhythm to resume at any moment.
But it didn't. The purple ball fibrillated and squirmed again, and the anaesthetist's head popped over the drapes to request the obvious – "Shock it again!" We did and the same thing happened. It wasn't coming back.
This was serious electrical instability caused by the scar tissue, so we gave the appropriate drugs to stabilise the muscle cell membranes.
"Let's give it more reperfusion time," I told the anaesthetist.
"OK, I'll go out for a fag then," he said.
Twenty minutes later we tried again. Twenty volts – zap! This time her whole little body levitated from the operating table and her heart defibrillated. Although it slowly began to beat, it was barely more than a flicker. Ominous, but we had drugs in reserve to make it pump harder.
I asked the anaesthetist to start an adrenaline infusion and told the perfusionist to cut back on pump flow to leave some blood in the heart. This was operating theatre protocol, and it's just like the military. You make a request to a medical colleague but give orders to the technical staff. If you start giving orders to an anaesthetist they'll tell you to piss off, and will go off and do something different.
My gaze remained fixed on Kirsty's pathetic little heart. The new coronary artery was fine – there was no kink in the tube and no bleeding. For the first time, the left ventricle was receiving well-oxygenated blood at the same pressure as the rest of the body. But her heart still looked like an overripe plum and was barely beating at all. What I was thinking was we're stuffed, this heart's had it; great operation – dead baby.
Of course, I didn't let the others know my thoughts but I was starting to fade. I suggested that the cameraman should stop filming for a while because nothing was going to change and asked Katsumata to come to my side of the operating table while I took a break.
I removed my gown and gloves, and went to make a call in the anaesthetic room. Mike followed.
"Can you repair it?" he asked me.
"Don't think so."
"I'll get Archer to warn the parents, then."
I slumped on a stool and picked up the phone. One of the lovely nurses put a coffee and doughnut in front of me.
In five minutes, Archer was down from his outpatient clinic at the theatre door. He didn't need to ask.
My bladder was full and I wandered off to the loo. When I got back, my brain had regained control without that distraction and I now really needed to focus. What, if anything, could I do to make things better? I was running out of ideas.
The left ventricle was scarred, dilated and now globular – not the normal elliptical shape. This distortion had pulled open the mitral valve and prevented it from closing. As the left ventricle tried to pump blood around the body as much as half of it flowed backwards to the lungs. Heart function is always temporarily worsened during surgery, but in Kirsty's case it seemed terminal. I'd hoped that resting the heart on the bypass machine would help it to recover. It hadn't.
I went back to the operating theatre, scrubbed up again and switched with Katsumata. He said nothing, but looked crestfallen – a clear message. I asked the anaesthetist to start ventilating the lungs and told the perfusionist to prepare to slowly ease off the machine. At this point, Kirsty's heart needed to take over and support the circulation, otherwise she'd die on the operating table. We all stared at the traces on the screen, hoping to see her blood pressure rise. It briefly reached half normal, but then fell away rapidly as the pump was switched off.
"Shall we go back on?" asked Katsumata.
Watching the left ventricle flicker on the echo, the perfusionist questioned whether it was worth it.
I wasn't yet ready to call it. Failure would mean death for the little girl and a life of torment for the parents.
"Let's go back on, give it another half hour."
This in itself was problematic, as a long bypass time always lessened the chance of recovery.
Kirsty's parents were waiting in the children's ward – Archer had gone to warn them. When we called him back, Becky insisted on coming to the doors of the operating theatre complex with him. Should I tell her that the heart was too badly damaged, that the diagnosis should have been made months ago and that Kirsty had been let down by an overburdened system?
On the very rare occasions that a child died on the operating table I always talked to the parents myself. It was something I dreaded, the very worst part of my job.
The entrance to the theatre complex opened automatically onto the hospital corridor. I was immediately confronted by eyes full of grief and desperation. I remember Becky saying, "Please save my little girl." I was pole-axed. I turned back to the sombre theatre, put on a new mask and scrubbed up again.
The anaesthetist had finished yet another cigarette, and said, "Things are no better. Can we turn the pump off?"
"No, I'm going to try one more thing. Turn the lungs off. Run the camera again."
This was my last-gasp attempt. It was something that could only be justified by invoking the laws of physics and had never been done before in a child. The tension on the wall of Kirsty's scarred left ventricle was elevated because of the size of the cavity. From a recent conference, I knew that a Brazilian surgeon had made a series of failing adult hearts smaller when a tropical infection, Chagas disease, had weakened the muscle. The operation had been attempted for other types of heart failure patients in North America but was quickly discredited and abandoned. In my view, this bold approach was Kirsty's last hope.
I was not going to risk stopping the heart again, so I took a glistening new scalpel and cut the beating left ventricle wide open from apex to base, just like unzipping a sleeping bag. I began in an area of scar, carefully avoiding the muscles that support the mitral valve, and the filleted heart immediately fibrillated in response to cutting. This was fine because there was no risk of it pumping air.
Frankly, I was stunned by the unexpected appearance of the inner lining of the heart. It was covered in thick, white scar tissue. To reduce the diameter of the ventricle I cut away the scar tissue on either side of the incision until I reached bleeding muscle and then removed one third of the circumference of the chamber.
In an attempt to stop the mitral valve from leaking I sewed the central point of its two leaflets together, turning it from an oval to a double orifice structure resembling a pair of spectacles. Then I simply sewed the muscle edges together with a double row of stitches to close the heart. In the end, this much smaller heart looked like a quivering black banana. Not for a moment did I think it would ever start again – and nor did my colleagues. Most of them thought I was crazy.
Word of the bizarre operation in Theatre 5 soon spread. The curious gathered to watch and the camera kept on filming. We had to ensure that all air was removed from the heart, otherwise it could be ejected into the blood vessels of the brain and cause a stroke. After that, all that remained was to defibrillate and try to restore normal heart rhythm.
"We're done," I announced. "Try 20 volts."
Zap! The heart stopped quivering, and for what seemed like an age there was no spontaneous electrical activity. I poked the muscle with forceps and it contracted in response. This time there was a flicker on the blood pressure trace. Miraculously, the black banana had ejected blood into the aorta.
The anaesthetist looked again at the echo. "It certainly looks different. Shall we try using the pacemaker?"
I was already sewing the fine pacing wires into place. We arbitrarily set the pacing box rate at 100 beats per minute and switched it on. I told the perfusionist to cut back on the pump flow rate and leave blood in the heart to see if it would eject consistently. It did. What's more, the echo showed that the mitral valve no longer leaked. At this point I felt that we were in with a chance. Life really does depend upon physics and geometry.
It was now after midday. Kirsty had been on the bypass machine for more than three hours and we needed to get her off it. As if timed to perfection, her own heart rhythm suddenly broke through the pacing. Coordinated natural heart rhythm is much more efficient than electrical pacing, providing much better blood flow and pressure.
It was like switching on a light in the operating theatre. Gloom changed to elation, adrenaline kicked in and my fatigue suddenly lifted. We gave Kirsty an infusion of adrenaline to help her heart take over from the bypass machine. Finally I gave the instruction to"'come off slowly". We still expected her blood pressure to fade, yet the curiously reconfigured little heart kept on pumping.
I remained silent, but I looked over my mask at Katsumata. He knew that I'd had enough by now. "Let me finish," he said.
I took a last, disbelieving glance at the little black banana pumping away, then turned to the echo screen, where the incomprehensible flashes of white, blue and yellow were also reassuring, like a blazing fire. We could see blood streaming through the new left coronary and a double jet entering the left ventricle through the mitral valve – a curiously reconfigured baby's heart that finally worked.
Katsumata made certain that there was no bleeding, then meticulously closed the chest.
"Never been done before," he said, looking over at me. Soon afterwards Becky came down to the theatre in shock. After the encounter at the theatre door, she believed Kirsty had died. She put her hand on her baby's little foot and exclaimed, "It's warm. It's never been warm." When she started to cry I left. It had been a long day.
We followed Kirsty carefully over the following ten years, using echocardiography to watch her heart develop. She was a perfect little girl, happy, outgoing and energetic, the only clue to her extraordinary internal metamorphosis being the faint stripe up the middle of her chest.
When we felt she was mature enough to discuss it, we asked for her permission to carry out a magnetic resonance imaging scan to show us how the remodelled heart had developed. What we found was quite extraordinary. Apart from the double-orifice mitral valve her heart appeared normal, as did her new left coronary artery. Only the thin nest of scars showed the position of the line of stitches up the heart. Remarkably, all other scar tissue had disappeared. The whole inner lining of her left ventricle had been pure white scar tissue – now all gone.
This provided some of the first evidence that an infant's own cardiac stem cells can regenerate heart muscle and actually remove fibrous tissue. Adult hearts cannot recover in the same way. But what if we could identify and culture stem cells that could do just this for an adult heart? One day I'd find out.
Now aged eighteen, Kirsty is a vivacious and athletic teenager. Had she died we'd never have known about this exciting possibility for heart regeneration.
This is an edited extract from Fragile Lives by Stephen Westaby, published by HarperCollins, available from February 20, RRP$32.99.
