Fiore S. Iorio

Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience.

BACKGROUND Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. METHODS Thirty-one patients (19.9 +/- 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. RESULTS There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. CONCLUSIONS Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.

Shelhigh No-React porcine pulmonic valve conduit: a new alternative to the homograft☆

BACKGROUND The Shelhigh No-React pulmonic valve conduit is a new porcine conduit that is glutaraldehyde-treated and detoxified using a proprietary heparin process. In our institution it has been implanted in 25 patients. The aim of this present contribution is to evaluate the short-term follow-up after its implantation. METHODS From November 1997 to August 1999, 25 patients (mean age, 20.2 years; range, 0.6 to 28.3 years) were operated on using this conduit. Seventeen patients underwent a Ross procedure for aortic valve disease, with the conduits implanted in anatomic position; 6 patients underwent right ventricular outflow tract reconstruction; 2 patients underwent the Rastelli operation. The follow-up was complete. Preoperative and postoperative two-dimensional echocardiography data were collected. RESULTS There were two non-conduit-related deaths. Two conduits needed to be exchanged because of an increase in the gradient. Overall, all patients were improved in terms of New York Heart Association class. Comparison of preoperative and postoperative two-dimensional echocardiography gradient showed significant improvement. At the 30-month follow-up, no calcification was seen on the explanted conduits or on the two-dimensional echocardiography, although many of the patients are children. CONCLUSIONS The Shelhigh conduits seem to be an alternative to homograft especially in infants. These experiences are preliminary, and longer follow-up is required.

Impact of DEL22q11, trisomy 21, and other genetic syndromes on surgical outcome of conotruncal heart defects

OBJECTIVE Genetic syndromes occur in more than 20% of patients with conotruncal heart defects. We investigated the impact of genetic syndromes on the surgical outcome of conotruncal anomalies in infancy. METHODS This retrospective study reviews the outcome of 787 patients (median age 6.3 months) who underwent primary (598) or staged (189) repair of a conotruncal defect between 1992 and 2007. RESULTS Proven genetic syndrome was diagnosed in 211 patients (26.8%), including del22q11 (91 patients), trisomy 21 (29 patients), VACTERL (18 patients), and other syndromes (73 patients). Primary repair was accomplished in 80.9% of nonsyndromic patients and 74.4% of syndromic patients (P = .18) Fifteen-year cumulative survival was 84.3% +/- 2.3% in nonsyndromic patients and 73.2% +/- 4.2% in syndromic patients (P < .001). Primary and staged repair allowed similar 15-year survival (81.4% +/- 4.5% vs 79.1% +/- 5.1%, P = .8). Freedom from noncardiac cause of death was significantly lower in syndromic patients (P = .0056). Fifteen-year Kaplan-Meier survival was 87.6% +/- 3.9% for del22q11, 95.8% +/- 4.1% for trisomy 21, 56.8% +/- 6.3% for VACTERL, and 62.3% +/- 12.7% for patients with other syndromes (P = .022). Total intensive care unit stay was 10.8 +/- 4.9 days in syndromic patients and 5.1 +/- 1.7 days in nonsyndromic patients (P < .001). Freedom from reintervention 15 years after repair was 79.6% +/- 4.9% in nonsyndromic patients and 62.4% +/- 7.4% in syndromic patients (P = .007). CONCLUSION Del22q11 and trisomy 21 do not represent risk factors for mortality after repair of conotruncal anomalies, whereas other syndromes adversely affect the surgical outcome for predominant noncardiac attrition. Higher morbidity and lower mid-term freedom from reintervention can be predicted in syndromic patients.

Papillary muscle rupture and pericardial injuries after blunt chest trauma.

Non-penetrating cardiac trauma resulting in mitral valve rupture is uncommon, requiring a high degree of suspicion for diagnosis. Sudden and severe mitral regurgitation, unless surgically corrected rapidly lead to congestive heart failure and death. We report a patient with traumatic rupture of the antero-lateral papillary muscle of the mitral valve and pericardial injury, after a lateral blunt chest trauma, who successfully underwent emergency mitral valve replacement.

Late Results of Extracardiac Fontan Repair

The Fontan procedure by means of an extracardiac conduit was initially proposed for patients presenting with anomalies of intra-atrial anatomy. We extended this technique to all patients with functional anatomic single ventricle. Between 1988 and 1998, 206 patients with complex cardiacanomalies underwent a total extracardiac cavopuolmonary connection. In 202 patients we used a conduit, in 4 patients we performed an IVC to pulmonary artery direct anastomosis. Ten patients underwent Conversion of a filing atriopulmonary Fontan procedure to a total extracardiac cavopulmonary connection. Early deaths occurred in 10% of patients and the extracardiac conduit was taken down in 3 additional patients. The cause of death was myocardial failure in 13 patients. Pulmonary distortion or hypoplasia in 6. No deaths have occurred in our last 45 patients. We observed no cases of conduit obstruction and thrombosis. Arrthymias were present in 16 patients. Ten patients underwent conversion of a formed modified Fontan. There were no immediate postoperative deaths. These results demonstrate that the total extracardiac cavopulmonary connection provide good early and mildterm results and is the technique of choice for a Fontan type repair. Copyright 1999 by W.B. Saunders Company

Total cavopulmonary direct anastomosis: a logical approach in selected patients.

A 2.5-year-old boy with a diagnosis of situs solitus, tricuspid atresia, anatomically corrected malposition of the great arteries (S,D,L), left juxtaposition of atrial appendages, and pulmonary stenosis underwent successful total cava-to-pulmonary connection by means of a superior vena cava-to-pulmonary artery end-to-side anastomosis associated with an inferior vena cava-to-pulmonary artery direct anastomosis. Anatomic features and surgical technique are described.

Transannular patching is a valid alternative for tetralogy of Fallot and complete atrioventricular septal defect repair

OBJECTIVE We report our experience with repair of tetralogy of Fallot associated with complete atrioventricular septal defect, addressing in particular the need for a pulmonary valve in the right ventricular outflow tract. METHODS Between 1992 and 2006, 33 children with tetralogy of Fallot and complete atrioventricular septal defect were admitted; 26 had Downs syndrome (79%). Thirty-two children had complete repair (18 primary, 14 staged); of the 15 who received initial palliation, 1 died before complete repair. Right ventricular outflow tract obstruction was relieved by transannular patch in 14 cases (42%), infundibular patch with preservation of the pulmonary valve in 7 (21%), and right ventricle-to-pulmonary artery conduit in 11 (33%). RESULTS There were no hospital deaths. Actuarial survival was 96% +/- 3.9% at 5 years and 85.9 +/- 1.1% at 10 years. Multivariate analysis showed that type of relief of right ventricular outflow tract obstruction did not influence survival (P = .16), nor did the choice to use a valved conduit (P = .82). Primary correction (P = .05) and lower weight at repair (P = .05) were associated with higher probability of survival. Mean follow-up was 69.3 +/- 5.9 months (range 0.2-282 months). There were 2 late deaths. Overall freedom from reoperation was 69% at 5 years and 38% at 10 years. Right ventricular outflow tract reconstruction without use of a valved conduit allowed a significantly higher freedom from reinterventions (P < .05). CONCLUSIONS Tetralogy of Fallot associated with complete atrioventricular septal defect can be corrected at low risk with favorable intermediate survival. Use of right ventricle-to-pulmonary artery conduit can be avoided in two thirds of patients with no impact on survival, possibly improving overall freedom from reintervention.

Morphology of the atrioventricular valve in asplenia syndrome: A peculiar type of atrioventricular canal defect

The high risk of atrioventricular (AV) valve regurgitation is a major point of concern in the natural history of patients with atrioventricular canal defect (AVCD) and asplenia syndrome (AS). The morphology of the common AV valve in patients with AVCD and AS was therefore studied and compared with the anatomy of patients with AVCD but with atrial situs solitus. We compared the anatomic features of hearts with common AV valve (ventricular loop; AV valve alignment on ventricles; number of leaflets; number and morphology of papillary muscles and relationship of the bridging leaflets with the ventricular septum) in the hearts of 33 patients with AVCD and asplenia syndrome (Group 1) with those of 44 patients with common AV valve, complete AVCD, and atrial situs solitus (Group 2). Hearts featuring asplenia syndrome showed a significantly higher occurrence of anomalies of the ventricular loop (p < .0001), right ventricular dominance (p < .01), and a reduced number of valvular leaflets and papillary muscles (p < .0005). On the contrary, hearts with situs solitus of the atria showed a significantly higher frequency of balanced ventricles (p < .0001) and a common AV valve with five leaflets and five papillary muscles (p < .000001). These data suggest that AVCDs in association with asplenia syndrome present morphologic peculiarities that may be considered as the substrate for the abnormal regurgitation. Recognizing the presence of these features could be of great value for the planning of a specific surgical treatment. These anatomic differences support the hypothesis of a genetic heterogeneity of AVCDs.

Reversal of protein-losing enteropathy after ligation of systemic-pulmonary shunt

A 21-year-old white woman, born with a univentricular heart, had undergone staged procedures before Fontan correction. She then began to develop edema, protein-losing enteropathy, and ascites refractory to diuretic therapy. Cardiac angiography showed a patent right Blalock-Taussig shunt, with turbulent cavopulmonary circulation. After undergoing an unsuccessful attempt at coil embolization she then underwent shunt ligation, with resolution of symptoms and normalization of protein levels. This report draws attention to the importance of cavopulmonary laminar flow to prevent the development of protein-losing enteropathy.

Neonatal pulmonary autograft implantation for cardiac tumor involving aortic valve

We diagnosed in a 4-day-old neonate a cardiac tumor involving the left atrium, left atrioventricular junction, left ventricular outflow tract, and aortic valve with severe subvalvular and valvular aortic stenosis. The critical involvement of the aortic valve and the scarcity of neonatal cardiac donors led us to perform a successful replacement of the aortic root with a pulmonary autograft, using a very small homograft for the native pulmonary valve (Ross operation).