Maurizio Rubino

Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases.

To facilitate the preoperative diagnosis and surgical management of visceral heterotaxy and asplenia, 72 postmortem cases were reviewed with particular attention focused on the systemic and pulmonary venous connections. The superior vena cava was bilateral in 51 cases (71%), but in 9 cases one of the superior venae cavae was partly or totally atretic. Patent bilateral superior venae cavae were found in 42 cases (58%) and the superior vena cava was unilateral in 21 (29%). Although the inferior vena cava was never interrupted, a prominent azygos vein was found in 6 cases (8%). Some hepatic veins drained separately from the inferior vena cava in 20 cases (28%). An intact coronary sinus was rare (2 cases, 3%). Anomalous pulmonary venous connection to a systemic vein was total in 42 (58%) of 72 and partial in 2 (3%) of 72, with obstruction in 24 (55%) of 44. Abnormal pulmonary artery branches (severe hypoplasia, localized stenosis, or discontinuity) were present in 21 (29%), and these obstructive arterial anomalies were associated with a significantly higher prevalence of anomalous pulmonary venous connection (p < 0.01) and of pulmonary venous obstruction (p < 0.01). Cardiac pulmonary venous connections were found in 28 (39%), with the pulmonary veins and the inferior vena cava entering the same atrium in 10 (36%) of 28.

Surgical excision of intracardiac myxomas: A 20-year follow-up

Since November 1968, 54 patients have undergone excision of an intracardiac myxoma, which was located in the left atrium in 46 (85%), in the right atrium in 6 (11%), and in the right ventricle in 2 (4%). There were 35 female and 19 male patients with a mean age of 48 +/- 14 years (range, 7 to 68 years). Four patients were asymptomatic; the others were seen mostly with exertional dyspnea, palpitation, signs of systemic illness, and syncopal episodes. Before operation, embolic episodes occurred in 13 patients with a left atrial myxoma. There were two early (3.7%) and two late deaths (3.8). Actuarial survival at 20 years is 91% +/- 4%, and most of the current survivors are asymptomatic at a mean follow-up of 6.5 +/- 5 years (range, 0.2 year to 20 years). Noninvasive reevaluation was performed with echocardiographic studies in 44 patients and 24-hour electrocardiographic monitoring in 34. No instances of tumor recurrence were observed, and there was a low incidence of major supraventricular arrhythmias late postoperatively. We conclude that excision of intracardiac myxomas is curative and long-term survival is excellent. The transseptal approach provides adequate exposure and allows complete removal of the tumor regardless of its location.

Repair of tetralogy of Fallot in the first six months of life: Transatrial versus transventricular approach

BACKGROUND This report describes our experience with primary correction of tetralogy of Fallot in infants. METHODS Fifty-one consecutive infants younger than 6 months underwent primary correction of tetralogy of Fallot between January 1978 and October 1994. Mean age at repair was 4.2 months. Four were neonates. Correction was accomplished through a right ventriculotomy in the first consecutive 22 patients (43%; group A); since 1991, a combined transatrial-transpulmonary approach was used in 29 consecutive patients (57%; group B). A transannular patch was necessary in 33 infants (65%) 16 of group A (73%) and 17 of group B (59%). RESULTS There was one early death from possible left anterior descending coronary artery distortion in group A and no deaths in group B. Two patients required early reoperation for systemic-to-pulmonary artery collateral ligation (postoperative day 6) and permanent pacemaker implantation (postoperative day 30). There were no late deaths. All 50 survivors are currently asymptomatic and in New York Heart Association class I. Three patients required late reoperations 36 months, 30 months, and 13 months after repair for (1) subaortic stenosis and dysfunctioning dysplastic mitral valve, (2) residual pulmonary artery branch stenosis, and (3) residual right ventricular outflow obstruction. Four patients underwent balloon dilation and stent insertion (1 patient) for peripheral pulmonary artery stenosis 1.5 year to 12 years (mean, 5 years) after initial repair. Actuarial freedom from need for reintervention at 4 years was 78.4% in group A and 85.7% in group B. Two-dimensional and Doppler echocardiographic follow-up studies showed a residual mild to moderate pulmonary artery branch stenosis in 4 patients in group A, and a recurrent subaortic stenosis in 1 patient in group A. Right ventricular peak systolic pressure was less than 40 mm hg in all but 3 asymptomatic patients who had a residual pulmonary artery branch stenosis. Right ventricular end-systolic and end-diastolic volumes showed larger volumes and reduced ejection fraction in group A compared with group B. CONCLUSIONS This limited experience with repair of tetralogy of Fallot in patients less than 6 months of age demonstrates that the transatrial-transventricular approach is possible in neonates and young infants with a very low mortality and morbidity and also a low incidence of residual lesions. Follow-up echocardiographic data suggest that right ventricular function is better preserved in those patients who underwent the transatrial-transpulmonary repair.

Surgical closure of apical ventricular septal defects through a right ventricular apical infundibulotomy.

BACKGROUND We present a new understanding of the anatomic position of apical ventricular septal defects and its surgical relevance. These defects occur between the left ventricular apex and the infundibular apex, rather than between the left and right ventricular apices. Often a sizable apical recess, the infundibular apex lies anteriorly and inferiorly to the moderator band and is the most leftward part of the right ventricle. METHODS Four patients (2 boys and 2 girls) with a mean age of 109 days (range, 48 to 217 days) underwent patch closure through an apical infundibulotomy, which allowed complete visualization of the muscular apical ventricular septal defect. RESULTS There were no early or late deaths at operation. No significant residual shunt at ventricular level was detected by postoperative two-dimensional and Doppler echocardiography. Intraoperative comparison of right atrial and pulmonary arterial blood samples showed a difference of less than 5%. At a mean follow-up of 18 months, all the patients are asymptomatic and growing well. CONCLUSIONS The successful outcome of these 4 patients indicates that surgical closure of apical ventricular septal defects can be achieved safely and completely in early infancy through a limited right ventricular apical infundibulotomy. Long-term follow-up of these and similar patients is needed to provide further evaluation of this approach.

Repair of congenital mitral valve dysplasia in infants and children: is it always possible?

OBJECTIVES Surgical management of congenital malformation of the mitral valve (MV) in the pediatric age group remains a therapeutic challenge for the wide spectrum of the morphological abnormalities and the high incidence of associated cardiac anomalies. We reviewed our experience so as to assess whether MV conservative surgery is always advisable and its results are superior to MV replacement. METHODS Thirty-four consecutive children (20 male and 14 female) with a mean age of 5.9 years (range 45 days-18 years) treated surgically for congenital MV disease between January 1987 and June 1999. Four patients (11.7%) were under 12 months of age, while 21 patients (62%) were younger than 5 years. Twenty-two patients presented with MV incompetence (or prevalent incompetence), while 12 presented with stenosis (or prevalent stenosis). Associated cardiac lesions were present in 22 patients (62.8%). RESULTS Mitral valve reconstruction was possible in all. There were no operative deaths. Three patients required reoperation for MV restenosis (a re-repair in one and MV replacement with mechanical prosthesis in two) 4 months, 27 months and 5.6 years after repair with no operative deaths. There was only one late death for prosthetic valve thrombosis. Follow-up data reveal that the 33 surviving patients are asymptomatic and well 4 months-12 years (mean 72 months) after surgery. At 12 years, actuarial survival and freedom from reoperation are 96.8 and 85.9%, respectively. Echocardiography performed in all of them shows no or mild incompetence or stenosis in 26 (78%), while residual moderate MV incompetence persists in six. CONCLUSIONS Our experience indicates that MV reconstructive procedures in infants and children with congenital MV dysplasia may be effective and reliable with low mortality and low incidence of reoperation rate. Mitral valve repair should always be attempted, especially in infants, despite the frequent severity of MV dysplasia, to avoid the drawbacks of the currently available prostheses.

Surgical treatment of complete A-V canal defects in children before 3 months of age

OBJECTIVES Surgical repair of complete A-V canal defects (CAVCD) is a well established procedure which is currently performed in infancy. The aim of this study is to evaluate surgical results of correction in early infancy in comparison to older age. METHODS From January 1985 to March 2001, 119 consecutive patients (age range 27 days to 83 months, mean 6.7 months) underwent repair of CAVCD in our Institution. Forms with unbalanced ventricles in association with Fallots tetralogy or heterotaxia were excluded from this series. Fifty-eight patients (49%) underwent correction before 3 months of age (Group A), and 61 patients (51%) after 3 months (Group B). Surgical repair was accomplished with a double patch technique in 100 patients (84%). Associated surgical lesions were treated simultaneously in 48 patients (40%). RESULTS There were 11 operative deaths (<30 days) (two in Group A (3.4%) and nine in Group B (15%)) (P = 0.05). The remaining patients were discharged home in good haemodynamic condition. Reoperation for postoperative left A-V incompetence occurred in five patients in Group A and in eight patients in Group B. There were eight late deaths (three in Group A and five in Group B), of which four were non-cardiac related. At a mean follow-up time of 80 months (range 2-184 months) 100 patients are asymptomatic and well, and free from oral medication. Echocardiographic examination showed absent or mild residual left A-V valve incompetence in 91 patients (49 in Group A and 42 in Group B) and moderate left A-V valve incompetence in nine patients (four in Group A and five in Group B). Kaplan-Meier survival estimates at 10 years were 90% for Group A and 75% for Group B. Kaplan-Meier freedom from reoperation at 10 years was 89% for Group A and 84% for Group B. CONCLUSIONS Our data demonstrate that repair of CAVCD under 3 months of age is the ideal approach to this malformation with a lower mortality rate at operation compared to older patients. Logistic analysis showed that an operative age >3 months is, compared to an age < or =3 months, an incremental risk factor for hospital mortality with an odds ratio of 4.8 (95% confidence limit 1-23.5) (P = 0.05). In the long term, freedom from reoperation for left A-V valve incompetence is higher when compared to children repaired at an older age.

Anomalous origin of one pulmonary artery from the ascending aorta

UNLABELLED We describe the surgical repair in three infants presenting with one pulmonary artery arising from the ascending aorta, the other artery arising normally from the right ventricle via the pulmonary trunk. Repair consisted of reimplantation of the anomalous pulmonary artery to the pulmonary trunk, in association with repair of associated intracardiac malformations. All patients survived the surgical procedures, and were discharged in stable clinical condition. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and underwent reoperation. Although survival after operation is now expected for this malformation, reports of late results are lacking. Larger numbers of operations are needed before we can reach definitive conclusions. The origin of one branch pulmonary artery from the ascending aorta in the presence of a pulmonary valve and main pulmonary artery is a very rare congenital cardiac anomaly. PATIENTS AND METHODS Between January 1995 and June 2003, 3 infant girls presented with the origin of one branch artery from the ascending aorta, while the other pulmonary artery originated from the pulmonary trunk which was in continuity with the right ventricular outflow tract. The pulmonary artery that arose from the right ventricle was left in 2 and right in 1 patient. RESULTS At the age 13, 48 and 62 days respectively, the patients underwent surgical repair consisting with reimplantation of the anomalous pulmonary artery branch to the pulmonary trunk in association with repair of intracardiac malformations. There were no hospital deaths. Postoperative complications included: prolonged intubation in two patients (10 and 16 days), low output syndrome in 1 patient, cardiac tamponade in 1 patient and seizures in 1 patient. All patients were discharged in good clinical condition. There have been no late deaths. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and after unsuccessful balloon dilation, underwent surgical reoperation. CONCLUSIONS Although operative survival is now possible for this malformation, reports of late results are lacking. Two of the three patients developed stenosis at the surgical anastomosis relatively early after surgery. Larger numbers of operations are necessaries to reach definitive conclusions.

Dehiscence of aortic valve prostheses: analysis of a ten-year experience

Up to the end of 1982, reoperation for dehiscence of an aortic prosthesis was necessary in 5% of patients operated on for primary aortic valve replacement in the previous decade at the University of Padova Cardiac Surgery Center. This complication occurred early (median time to diagnosis 4 months) and was associated with an elevated 30-day operative mortality (27%, 70% CL 19-37%). This is probably (P = 0.09) related to preoperative heart failure. The follow-up of the traced surviving patients (92%) indicates a continuing poor prognosis with a 6-year survival rate of only 13.5% (70% CL 5.0-30%). All the events were cardiac related and directly or indirectly connected with the persistence or recurrence of dehiscence that was observed in 72% of the cases. In the face of these results, a retrospective study has been performed to identify, on the basis of the available data, the subsets of patients more prone to develop this complication. Our results suggest that a significant increased risk (P less than 0.001) can be identified in patients presenting with bacterial endocarditis (12.2% rate), in patients with concomitant aneurysm of the ascending aorta (10.9%) and in patients with degenerative regurgitation or severe calcifications of their native valve, with rates of 7.0 and 6.0 respectively. In these situations particular care is required to avoid undue stress on the annular tissue. We also suggest the use of buttressed interrupted sutures.

Early and late survival of repaired type A aortic dissection

From 1 January 1977 to 31 December 1988, 119 patients were operated upon for type A aortic dissection. The maximum follow-up was 11.7 years (median 5.6 years); follow-up was 100% complete. Actuarial survival was 47.3% +/- 5%. The death risk decreased rapidly to a constant rate of 0.0027 events/month after 3 months. There were 41 early deaths, mostly due to haemorrhage, brain damage and low output syndrome. A significantly higher probability of early death was observed in patients with preoperative myocardial ischaemia or infarction (P less than 0.0001) or preoperative cerebral symptoms (P = 0.0002). Extended dissection increased the risk proportionally to the length of the aorta involved (P = 0.0002). Typical dissection originating from an intimal tear in the ascending aorta had a significantly lower operative risk than atypical dissection with an intimal tear not localized in the aortic root (P = 0.0006). Of the 14 late deaths, 2 were unrelated to dissection, 2 were of unknown origin and 4 were sudden. Stroke was the cause of 2 and congestive heart failure the cause of 4 deaths. The probability of late death was higher in patients with perioperative brain damage (P = 0.003) and in patients with preoperative shock (P = 0.0025). It was significantly lower in patients with dissection of hypertensive aetiology (P = 0.002). There were 13 reoperations on 12 patients. Early reoperations were due to rupture of the distal aortic anastomosis. Late reoperations were mostly due to dehiscence of aortic valvular prosthesis.(ABSTRACT TRUNCATED AT 250 WORDS)

Giant congenital aortic aneurysm with cleft sternum in a neonate: pathological and surgical considerations for optimal management

We report a rare case of a neonate with congenital giant aortic aneurysm associated to cleft sternum, who underwent surgical repair. The patient died on postoperative Day 5 from cardiac arrest. Autopsy revealed a circumferential subendocardial myocardial infarction and misdiagnosed coronary ostial anomalies. A critical analysis of this unfortunate case may help optimal surgical planning in similar patients in the future.