History of cardiac and thoracic surgeryA cardiac surgeon, his way to holiness, and his heritage – the 80th anniversary of Giancarlo Rastelli’s birthday and the 45th anniversary of the first Rastelli procedure (2024)

Introduction

In 1969, a12-year old boy, Vincenzo Ferrante, arrived in theUSA (Houston, Texas) with his parents. He was supposed to be operated on by surgeons from Texas, since theprevious surgical procedure that he had undergone (atrioseptectomy, i.e. theBlalock-Hanlon procedure) 3 years before was performed there as well. Unfortunately, it turned out that thelevel of thedefect’s complexity (transposition of thegreat arteries with ventricular septal defect and pulmonary valve stenosis) disqualified thepatient from further treatment. Thedesperate parents turned for help to Doctor Giancarlo Rastelli. Thecardiac surgeon, himself struggling with Hodgkin’s lymphoma for almost ayear, decided to diligently treat his compatriot. Theboy underwent two operations at theMayo Clinic in Rochester in November 1969. Thefirst was theRastelli procedure, but, unfortunately, reoperation was necessary due to therecanalization of ventricular septal defect. Thecondition of theyoung Italian was improving each day, while thehealth of Doctor Giancarlo Rastelli was gradually deteriorating. In spite of this, thephysician visited his patient every day. He even spent Christmas Eve next to his bed, because the12-year-old had to stay at theclinic for theholidays. Vincenzo Ferrante left thehospital after several weeks and returned to Italy with his parents. He went to university, became an engineer, working in Naples. Giancarlo Rastelli died less than amonth after theboy was discharged from thehospital on February 2, 1970. He was 36 years old. He died at thepeak of his professional career. Theprocess of his beatification in theVatican has lasted since 2005... [1, 2].

Who was theman whom modern doctors working with congenital heart defects usually associate with such eponyms as theRastelli classification and theRastelli procedure? What were theextraordinary circ*mstances that may result in theunusual event that for thefirst time in history thewords “blessed” and, later on, “saint” may appear before thename of acardiac surgeon?

Giancarlo Rastelli was born 80 years ago on June 25, 1933 in thetown of Pescara, Italy. His father, Vito Rastelli, was ajournalist for alocal newspaper, and his mother, Luisa Bianchi, worked as aprimary school teacher. Giancarlo also had ayounger sister, Rosangela. His uncle (Lino Rastelli) was afamous pianist. Having graduated from high school at theage of 17, Giancarlo was accepted into themedical faculty at theUniversity of Parma. He developed an interest in surgery during thethird year of his studies. Later on, he narrowed his focus to cardiovascular surgery. He graduated from theUniversity of Parma with distinction in 1957, receiving acum laude diploma for his work entitled: Changes in theATPase activity of themyocardium during deep hypothermia. Three years later, Giancarlo Rastelli became aNATO scholarship holder, which enabled him to choose one of several centers in theUSA. He chose theprestigious Mayo Clinic in Rochester, where one of thepioneers of cardiac surgery, John Kirklin (1917-2004), worked. Initially, Rastelli focused on invasive cardiac surgery, exploring thepathom*orphology and pathophysiology of thecirculatory system at thelaboratory of cardiac catheterization and angiocardiography, supervised by Jeremy Swan. In this manner he established thebasis for his experiments in cardiac surgery. In 1962, Rastelli started assisting at his first cardiac surgery procedures. John Kirklin – his mentor and teacher – wrote years later: Ipersonally have learned many things from Dr. Rastelli. In thenumerous scientific projects that we worked on together, new knowledge was developed which has been of both practical and theoretical importance to me [1]. Rastelli was especially interested in one congenital heart defect: atrioventricular septal defect (AVSD), thecorrection of which was associated with avery high mortality rate. As aresult of his pathom*orphological research conducted on alarge number of dissected hearts, in 1967 he published anew classification of AVSDs [1-4].

TheRastelli classification

TheRastelli classification is based on themorphology of thesuperior bridging leaflet of thecommon atrioventricular canal and its relation to theinterventricular septum and thepapillary muscle of theright ventricle.

In type A(50-70% of all AVSD cases), thesuperior bridging leaflet is symmetrically medially divided along theline of theinterventricular septum, with its two parts, left and right, attached with multiple chordae tendineae to theinterventricular septum in their respective ventricles. Theleft side of thecommon valve is displaced towards theapex and forms acommon annulus with theright side. Type Aoften coexists with Down syndrome and is usually associated with pulmonary hypertension. In type B (3% – therarest of thetypes), thesuperior leaflet is asymmetrically divided in such away that its left component is partially displaced above theright ventricle, and thechordae tendineae cross theventricular septal defect reaching thepapillary muscle of theright ventricle. It is usually accompanied by thelack of one left ventricular papillary muscle, and themitral part of thecommon valve may then form aparachute valve. Type B is often associated with unbalanced AVSD with right ventricular dominance. Type C of AVSD (30%) is characterized by an extreme displacement of theleft superior leaflet above theright ventricle, so that it is freely located above theventricular septal defect, and thechordae tendineae branching from it are attached to thepapillary muscles of both ventricles. This form often coexists with heterotaxy syndrome and with conal defects [5-7].

In 1967, Rastelli et al. published apaper presenting theexperience of theMayo Clinic in operating on patients with complete AVSDs (38 patients, 1955-1967). Thanks to thebetter understanding of this defect and theadjustment of treatment to thenew classification, theauthors managed to reduce mortality from 60% (operations before 1964) to 20%. They also concluded that thedivision of AVSD into three anatomical subtypes enabled abetter understanding of thenature of this defect and contributed to theimprovement of surgical treatment outcomes [8]. It was very important for Rastelli to have his book-monograph devoted to AVSD published. He had to work against time because of theprogression of his disease. Unfortunately, he did not manage to finish his work and left this task for others to complete. Themonograph was finally published in 1976 [2].

Today, theRastelli classification is no longer as useful as when it was first created. It did not take into account thenumerous anatomical variations of AVSD, because of which some surgeons rejected it altogether. Some believe that thedegree of leaflet restriction (tethering) is more relevant; therefore, in their opinion, theRastelli classification today is only of historical importance [6]. Others claim, however, that despite thediversity of theanatomical variants of AVSD, theclassic division of Rastelli still remains avery useful, clear, and effective tool in surgical practice [9].

There is also one more eponym related to AVSD containing thename of Giancarlo Rastelli, referring to asurgical technique used for repairing anomalies typical of this defect. This method, theRastelli procedure, was implemented in 1967. It was based on theuse of asingle patch covering both theatrial and ventricular septal defects. Themethod was later modified: Dacron or Gore-Tex patches were used for closing ventricular septal defects, while pericardial patches were employed for repairing atrial septal defects. Currently, in selected cases, amodified version of thesingle-patch technique is used, in which thevalve components are attached to theapex of theinterventricular septum. In 2011, theresults of astudy by French authors were published, which included theanalysis of 107 infants below theage of 1 with complete AVSD, operated on between 1985 and 2006 in Marseille. Theinitial total operative mortality rate of 13% dropped to 4% within thelast 10 years. Theauthors of thecited work stated that theRastelli procedure for repairing AVSD is safe and repeatable, and it provides satisfactory results [9].

TheRastelli procedure

In theyears 1962-1964, Giancarlo Rastelli conducted surgical experiments at theexperimental laboratory of theMayo Clinic. He was initially interested in persistent truncus arteriosus, aheart defect inoperable at that time. He attempted to close theventricular septal defect and use ahom*ograft to connect theright ventricle to thepulmonary trunk separated from thecommon trunk. His successful experiments encouraged Dwight McGoon to perform thefirst operation using this technique in 1967. Theuse of this innovative solution, and theapplication of avalve hom*ograft connecting theright ventricle with thepulmonary trunk combined with theintroduction of apatch directing blood flow from theleft ventricle through theventricular septal defect to theaorta, became thebasis for themethod currently known as theRastelli procedure. Originally, this procedure was used in treating transposition of thegreat arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO). Thefirst surgery using theRastelli procedure to repair this complex defect was performed at theMayo Clinic by Robert Wallace on July 26, 1968, precisely 45 years ago. Themethod was utilized ayear before during theworld’s first successful repair of persistent truncus arteriosus (also at theMayo Clinic, Dwight McGoon) [1, 2, 10, 11].

TheRastelli procedure is also currently used in other heart defects, including double outlet right ventricle with left ventricular outflow tract obstruction or atresia. Despite thefact that almost half acentury has passed since thefirst Rastelli procedure was performed, thesurgical treatment of patients with TGA, VSD, and LVOTO continues to pose achallenge. Thelong-term results of theRastelli procedure remain arelevant object of study. Thesynthetic material used in LVOTO reconstruction does not have any growth potential. Moreover, therisk of LVOTO increases with time because of thenatural tendency of VSD to gradually close, resulting in dysfunction of theleft ventricle and arrhythmias. In order to avoid LVOTO, modifications were introduced consisting in theexcision of theconal septum and widening of theVSD before establishing an outflow tract from theleft ventricle to theaorta by means of apatch. This improved theearly and mid-term survival after theRastelli procedure. Thelate stenosis of theright ventricle to pulmonary artery (RV-PA) conduit requiring reintervention still remains asignificant problem. Initially, thetreatment results of patients after theRastelli procedure were far from optimal. In 2000, Kreuzer et al. published apaper summarizing their 25-year long surgical experience with theRastelli procedure (1973-1998, 101 patients). After 20 years, 52% of thepatients survived and did not require aheart transplant [12]. This percentage was greatly improved thanks to thefurther advancement of operative technique, better myocardial protection, changes in surgery qualification criteria, and broader experience of cardiac surgeons. In 2011, apublication by American authors appeared concerning patients operated on between 1988 and 2008. Their 20-year survival rate amounted to 93%. Therisk factors for death or heart transplant included theperformance of theRastelli procedure before 1998 and thepresence of extracardiac anomalies. 40% of thepatients required reoperation due to RV-PA conduit stenosis. On thebasis of theconducted data analysis, it was established that in 59% of cases there was no need to replace theconduit after 20 years. Therisk factors for conduit replacement included young age of thepatients and operation before 1988 [13].

TheREV procedure

In 1982, Lecompte et al. proposed atechnique for right ventricular outflow tract reconstruction using apatch instead of avalve conduit (REV – Réparation à l’Etage Ventriculaire) as an alternative for patients who were candidates for theRastelli procedure [14]. Thekey part of this method is theexcision of theconal septum, which widens theVSD and prevents LVOTO. This technique also utilizes apatch which directs blood flow from theleft ventricle to theaorta. Theright ventricle is directly connected to thepulmonary trunk with aunicuspid valve or without it. This method is associated with higher early mortality in comparison to theRastelli procedure. Thepatients also require monitoring due to right ventricular outflow tract obstruction (RVOTO). Theyear of 2011 saw thepublication of awork originating from Rastelli’s homeland (written at theBambino Gesù Children’s Hospital in Rome). Theauthors (including Yves Lecompte) analyzed long-term results of 205 patients treated with theREV method between 1980 and 2003. 25-year survival was 85%; 45% of patients did not require any surgical procedure during that time. Themost common reason for reoperation was LVOTO. In 77% of patients, no arrhythmias were found; 87% were in NYHA class I. In their conclusions, theauthors stated that thepresented surgical outcomes with theREV method were more favorable than operations based on theRastelli procedure, both in terms of survival rate and thenecessity of reoperation because of left or right ventricular outflow tract obstruction [15]. TheREV method has not gained wide popularity so far, while theRastelli procedure still remains thepreferred option in themajority of cases.

TheNikaidoh procedure

In 1984, Nikaidoh presented another concept of surgical treatment for d‑TGA with VSD and LVOTO: aortic translocation with reconstruction of theoutflow tracts of both ventricles [16]. This method, which includes elements of theRoss, Jatene, and Konno procedures, consists in separating theaorta from theright ventricle, removing left ventricular outflow tract obstructions by intersecting theconal septum and excising thepulmonary valve, reconstructing theleft ventricular outflow tract leading to thetranslocated aorta with apatch appropriately sutured onto theventricular septal defect, and reconstructing theright ventricular outflow tract using apericardial patch. Modifications of this method include translocation of coronary vessels during aortic translocation (to prevent ischemia), theLecompte maneuver, and reconstruction of theright ventricular outflow tract with apulmonary hom*ograft or theestablishment of adirect connection of theright ventricle with thepulmonary artery [17, 18].

TheNikaidoh procedure – theperformance of which is atechnical challenge – is mainly recommended in thecases of atrioventricular canal type VSD (distant from LVOT) or restrictive VSDs, hypoplastic right ventricle, or straddling atrioventricular valve (thevalve “straddles” theinterventricular septum). In such situations, it may sometimes be theonly option for biventricular correction [17-19]. It is believed that abnormal distribution of thecoronary arteries constitutes acontraindication for theNikaidoh procedure, but, in 2011, thejournal Cardiology in theYoung published adescription of asuccessful operation with this technique performed in apatient whose anterior interventricular branch of theleft coronary artery crossed theright ventricular outflow tract [20].

In 2010, amulticenter paper was published comparing theresults of various methods used for thetreatment of TGA with VSD and LVOTO [19]. Theanalysis included 146 patients undergoing different surgical procedures in theyears 1980-2002 in eight European centers. In theintroduction, theauthors stated that thediscussion concerning thesurgical treatment of TGA with VSD and LVOTO is mainly focused on thecomparison of outcomes between theRastelli, Nikaidoh, and REV methods, and that there has never been published any paper that demonstrates theuniversal superiority of one of these procedures over theothers. 82 patients underwent theRastelli procedure (56.2%), theJatene method (arterial switch) was performed in 24 patients (16.4%), REV in 7 patients (4.8%), atrial switch in 5 patients (3.4%), and theNikaidoh procedure in 4 patients (2.7%). Theearly mortality rate was higher in thecase of atrial or arterial switch with theclosure of VSD and therelief of LVOTO. Thelate mortality rate was lower in thegroup of patients operated on using theREV technique and its modification with theMetras method. Reoperation due to RVOTO was most frequent in thecase of patients undergoing theRastelli procedure (37.7% of patients), followed by theREV method (14.3%) and arterial switch (13%). Thehighest percentage of patients not requiring reoperation or percutaneous intervention was found in thegroup undergoing theNikaidoh procedure, followed by REV and theMetras modification. Reinterventions were more common in thecase of LVOTO than of RVOTO. Themortality risk factors were theage of theoperated children, VSD “non comitted” to theaorta or pulmonary artery, and theduration of theprocedure (extracorporeal circulation). Thetype of theprocedure was not arisk factor for mortality. Theage of thepatients, year of theoperation, and type of theprocedure constituted significant prognostic factors with regard to thenecessity of reoperation or percutaneous intervention. Ahigher risk of reoperation was found in patients undergoing theRastelli procedure. In theREV method and its modification, conduit implantation is avoided and, thanks to theexcision of theconal septum, asimple connection between theleft ventricle and theaorta is achieved, which, unfortunately, does not exclude thepossibility of future reoperation due to LVOTO. In thecited work thenumber of patients undergoing theNikaidoh procedure was too small (4 children), and thefollow-up period was too short to draw any far-reaching conclusions. In conclusion, theauthors of theaforementioned multicenter publication claim that their analysis confirmed thepreviously reported less-than-optimal results of theRastelli procedure, which – in comparison to theREV method and its Metras modification – requires reintervention more often, especially due to RVOTO [19].

Rastelli, Nikaidoh, or REV?

Thesurgical treatment of TGA with VSD and LVOTO still remains achallenge because of thenecessity of reconstructing theoutflow tracts of both ventricles. There seems to be atendency to perform certain procedures in certain countries. TheREV technique of Yves Lecompte and its modification with themethod by Dominique Metras was initially widespread only in francophone countries, while theRastelli procedure was popularized all around theworld. Theyoungest of theused methods – theNikaidoh procedure – comes from Japan, but it originated in France (apublication by Jean-Pierre Bex and Yves Lecompte in 1979). It is believed that theanatomical effect ensured by theNikaidoh method is “closer to thenorm” and provides abetter location of theleft and right outflow tracts and alower risk of pressure exerted by thesternum on theright ventricular outflow tract in comparison to theRastelli procedure (non-anatomical branching of theconduit from theright ventricular outflow tract in theRastelli procedure). Abnormalities of coronary arteries constitute acontraindication for theNikaidoh method. Aposterior, intramural location of acoronary artery poses difficulties in translocating theaorta to theback, towards thepulmonary annulus. This type of anatomy may also prevent thetransplantation of coronary arteries in conjunction with aortic translocation. In such situations, theRastelli procedure seems to be abetter option [19-22].

TheCardiac Surgery Clinic at theChildren’s Memorial Health Institute in Warsaw is thelocation of theEACTS (European Association for Cardio-Thoracic Surgery) Congenital Database. Moreover, it also runs theNational Register of Cardiac Surgery. According to theEACTS data, in thefive best centers in theworld, themortality rate for operations performed using theRastelli procedure is 6.6% (15/225 patients), while for operations using theREV procedure it is 4.35% (5/115 patients). TheNikaidoh procedure was included in theEACTS database under the“other” category (together with, for example, theKawashima procedure); themortality rate of this group is 10.53% (6/58 patients).

TheRastelli, Nikaidoh, and REV procedures constitute three complementary options of surgical treatment. Each of these methods has its advantages and drawbacks, limitations and specific indications. There are patients who are better served by theNikaidoh procedure than theRastelli procedure. In thecase of restrictive VSD and theimpossibility of sufficient excision of theconal septum, theNikaidoh procedure is abetter option. TheREV technique has similar contraindications as theRastelli method. Each of these three options for surgical treatment has found aplace in cardiac surgery, and thechoice of an optimal method may sometimes be controversial with regard to mortality rates and long-term outcomes. However, for thevast majority of centers in theworld, theRastelli procedure still remains themethod of choice [19-22].

Epilogue

Between 1968 and 1969, Giancarlo Rastelli received two gold medals of theAmerican Medical Association – for his research on AVSD and for creating an innovative surgical technique for thetreatment of persistent truncus arteriosus and transposition of thegreat arteries. He did not want to renounce his Italian citizenship, but after spending 7 years in theUnited States, in accordance with US law, he had to accept American citizenship. Thesolution turned out to be aspecial decree issued by President Nixon especially for him. In 1959, before his trip to theUSA, Rastelli met Anna Anghileri, who was 19 at thetime, during askiing camp in theAlps. He wrote letters to her from America almost every day. Five years later, he took aflight to Europe, to Italy, and one day after arrival he married Anna (August 12, 1964). Soon after, Rastelli diagnosed himself with Hodgkin’s lymphoma. He was too intelligent not to know what it meant... [1-4].

Giancarlo Rastelli loved themountains and classical music – especially Verdi, Vivaldi, and Purcell. He had theappearance of aromantic lead and was liked by everyone. He was not able to help many children with complex heart defects. Still, he claimed that adoctor can always give these children something from himself, even if it is just several minutes of daily attention, avisit to their room, asmile, or ahandshake. He was very devoted to his patients – especially Italian children pilgrimaging with their parents from Europe in thehope that American doctors would fix their incorrectly built hearts. Dr Rastelli cared about these children, invited them home, fed them, and helped them collect thefunds necessary for theoperation. He had aposter in his office with l’amore vince (love conquers) written in Italian, on which he collected thesignatures of his Italian patients [2, 4, 23].

When he was losing his strength because of theprogression of his disease, his sister – Rosangela – advised him to slow down and rest. He did not want to, he refused. He wrote that not working meant not living for him. John Kirklin, Rastelli’s mentor and teacher, said thefollowing several days after his death: Perhaps themost remarkable aspect of this man’s life was his reaction to his fatal illness. About five years ago, he walked into my office and said that he had Hodgkin’s disease. He told me this with about thesame display of emotion that he would have used should he have said that our densitometer was not working properly. Somehow an unspoken agreement developed between us that neither of us would speak of this illness unless there was urgent need to do so. About ayear and ahalf later, another recurrence developed and he told me of this in thesame simple words. Theserenity and confidence with which he faced life and death is thegreatest of themany things that he taught me.” [1, 2, 23, 24].

Giancarlo Rastelli died in aRochester hospital after afive-year struggle with thedisease, 4 months before his 37th birthday. He orphaned his 4-year-old daughter, Antonella Luisa (she is currently adoctor and works in St Louis). His body was brought to Italy and buried in thechapel of theUniversity of Parma. 35 years later – on September 30, 2005 – his beatification process began in theVatican. Thelife of Rastelli was considered an example and inspiration for young Catholics, medical students, and novice physicians.

Those who know his biography believe that his attitude towards patients, life, and being adoctor is an example to follow. Themost important issue in thebeatification process is to prove theextraordinary nature of thelife of theperson who died in theaura of sanctity. It is related to theso-called “heroism of virtues” (heroicas virtutum). Thepetitioner of Rastelli’s beatification is theDiocese of Parma and theAssociation of Catholic Doctors. Thetask will be to prove, among other things, theheroism of Giancarlo Rastelli’s virtues. Thebeatification process is led by theCongregation for theCauses of Saints (protocol no. 2678). Time will show whether thecardiac surgeon will become asaint... [1-4, 23, 24].

Theauthors would like to sincerely thank Dr Antonella Rastelli from theWashington University School of Medicine in Saint Louis for providing and allowing us to publish thephotograph of her Father.

Theauthors do not declare any conflict of interests.

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