Marcelletti C

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.

Extracardiac Fontan operation for complex cardiac anomalies: Seven years' experience

METHODS Between 1988 and 1995, 60 patients with complex cardiac anomalies underwent a total extracardiac cavopulmonary connection, a combination of a bidirectional cavopulmonary anastomosis with an extracardiac conduit interposition between the inferior vena cava and pulmonary arteries, except in one patient in whom direct anastomosis was possible. In 40 patients the total extracardiac cavopulmonary connection followed preliminary bidirectional cavopulmonary anastomosis, associated with a modified Damus-Kaye-Stansel anastomosis in 16. The conduits were constructed of Dacron fabric (n = 34), homografts (n = 3), and polytetrafluoroethylene (n = 22). RESULTS Total early failure rate was 15% (n = 9). Six patients died, and three more had conduit takedown owing to pulmonary artery stenosis and hypoplasia (n = 2) and severe atrioventricular valve regurgitation (n = 1). Two other patients required anastomosis revision owing to stricture. In a mean follow-up of 48 months (6 to 86 months) there were no late deaths (actuarial 5-year survival 88% +/- 4%); 52 of 54 patients are in New York Heart Association class I or II. Two patients required pulmonary artery balloon dilation or stent implantation, or both, after total extracardiac cavopulmonary connection. Late tachyarrhythmias were detected in four of 54 patients: two had sick sinus syndrome with flutter necessitating a pacemaker implantation and two had recurrent flutter (actuarial 5-year arrhythmia-free rate 92% +/- 4%). Conduit patency was evaluated by serial magnetic resonance imaging studies. Preliminary data showed a 17.8% +/- 7.6% mean reduction in conduit internal diameter during the first 6 months after total extracardiac cavopulmonary connection, with no progression over the next 5 years. CONCLUSION These results demonstrate that the total extracardiac cavopulmonary connection provides good early and midterm results and may reduce the prevalence of late arrhythmias in patients undergoing the Fontan operation.

Usefulness of magnetic resonance imaging for diagnosis of acute myocarditis in infants and children, and comparison with endomyocardial biopsy

The diagnosis of acute myocarditis has traditionally been based on the results of endomyocardial biopsy.1 This invasive procedure, particularly in children, may present major complications and is performed only at a limited number of centers. Recent studies have demonstrated the reliability of magnetic resonance imaging (MRI) in tissue characterization of cardiac allograft rejection2–4 that is histologically identical to acute myocarditis.5 The aim of this study was to test the sensitivity and specificity of MRI versus endomyocardial biopsy in the diagnosis of acute myocarditis in children.

Right oblique subxiphoid view for two-dimensional echocardiographic visualization of the right ventricle in congenital heart disease.

Two-dimensional echocardiography in the right oblique subxiphoid view (ROSV) was used to visualize all right ventricular components in 351 patients younger than 2 years of age (100 normal and 251 with various types of congenital heart disease). The ROSV, which is anterior and parallel to the ventricular septum, displays all right-sided cardiac components, the left atrium and the aorta. This view provided the correct diagnosis in patients with pulmonary stenosis or atresia with intact ventricular septum, Ebsteins anomaly, tricuspid atresia, infundibular ventricular septal defect, tetralogy of Fallot and truncus arteriosus. In all patients studied with the ROSV, an excellent definition of morphologic characteristics, equivalent to that in the right oblique axial angiocardiographic view, was achieved. The ROSV should be used routinely to delineate the right ventricular anatomy in all infants with congenital heart disease.

Patient selection for repair of complete atrioventricular canal guided by echocardiography

Objective. Two-dimensional, Doppler and color-flow echocardiography is accepted as a safe diagnostic tool to guide the surgical treatment of certain congenital heart defects. Its role for surgical indication in patients with complete atrioventricular canal (cAVC) is described in this paper. Methods. Between July 1989 and January 1995, 80 patients with cAVC underwent primary repair in our Institution. In 73 surgery was guided only by echocardiography (Group I), while in the remaining 7 patients (Group II) cardiac catheterization was performed because of unclear diagnosis and/or suspected associated malformations. Results. Echocardiography showed an overall sensitivity of 100% and specificity of 93% in detecting associated malformations. Discussion. Based on these data and on the knowledge of the natural history of pulmonary vascular changes in patients with cAVC we think that echocardiography might be adequate for selection of infants with uncomplicated forms of cAVC for primary repair.

Complete transposition of the great arteries: Visualization of left and right outflow tract obstruction by oblique subcostal two-dimensional echocardiography

Subcostal oblique 2-dimensional echocardiography was performed in 64 infants younger than 2 years with complete transposition of the great arteries (TGA) (situs solitus, concordant atrioventricular and discordant ventriculoarterial connections). All patients examined before cardiac catheterization had a correct diagnosis by 2-dimensional echocardiography using the subcostal oblique views. Twelve patients had associated left ventricular (LV) outflow tract obstruction and 7 had right ventricular (RV) outflow obstruction. The standard parasternal views failed to diagnose obstruction in 1 patients with LV outflow obstruction and 5 with RV outflow obstruction; the subcostal left oblique cut and long axis of the left ventricle visualized all left-sided obstructions, and right-sided obstructions were correctly displayed in 5 of 7 cases using a combination of left oblique and right oblique cuts. Two-dimensional echocardiographic subcostal oblique views allow an excellent definition of the morphologic characteristics of RV and LV outflow tracts in patients with TGA and improve the diagnosis of the outflow obstruction in these malformations.

Palliative Mustard operation: an expanded horizon.

From March 1982 to December 1983, five patients with a mean age 7 years (4 months-16 years) underwent a palliative Mustard operation for complex cardiac anomalies. The diagnoses were: univentricular heart with pulmonary vascular obstructive disease (PVOD) (2 cases); criss-cross heart with intact atrial septum, ventricular septal defect (VSD), transposition of the great arteries (TGA) and moderate pulmonary stenosis; isolated atrioventricular discordance with VSD; TGA, diminutive right ventricle and multiple VSDs. Cardiac catheterization showed unfavourable direction of flow: the oxygen saturation in the aorta was lower than in pulmonary artery, with a mean difference of 13.5% (range 6% to 30%). In no case was anatomical or physiological repair considered advisable because of the unfavourable intracardiac anatomy or the presence of PVOD. In two patients the atrial rerouting was accompanied by pulmonary artery banding. There were no hospital or late deaths in a mean follow-up of 36 months (range 29 to 50 months). The mean post-operative arterial oxygen saturation was 95.4% (range 92% to 99%).

Early identification of patients at risk for sinus node dysfunction after Mustard operation

We studied 60 patients who had survived the Mustard procedure for transposition of the great arteries, performed between the ages of 2 days and 24 months (mean 4.51 +/- 3.79). All patients were given a postoperative 24-hour dynamic electrocardiogram at 15 days, 1 year and 3 years and then every 2 years. Those who were found to have sinus node dysfunction during follow-up, were given a dynamic electrocardiogram every 3-6 months. The average follow-up period was 38.7 +/- 19.8 months, median 36. Sinus node dysfunction was detected during follow-up in 20 patients (33.3% of the total), 8 of whom had had a pacemaker inserted. Risk factors for late development of sinus node dysfunction were found to include prolonged cross-clamping of the aorta during surgery (P = 0.003), especially over 50 minutes (relative risk 3.5:1), and the presence of even transient sinus node dysfunction after surgery (P = 0.006). These observations suggest, first, that sinus node dysfunction may develop after the Mustard operation as a combined effect of extensive atrial surgery and a long period of myocardial ischemia and, second, that the presence of the disease immediately after the operation sometimes indicates that it will recur or persist during follow-up.

Systemic atrioventricular conduit for extracardiac bypass of hypoplastic systemic atrioventricular valve.

The management of severe congenital mitral stenosis in infants and children is still controversial. We describe our experience with the use of a systemic atrioventricular (SAV) extracardiac conduit to bypass a hypoplastic systemic atrioventricular valve. An SAV extracardiac conduit has been used in six patients (left atrium--left ventricle in five, right atrium--right ventricle in one). One hospital death occurred due to mediastinitis and there were two late deaths, one due to progressive subaortic stenosis and one sudden, possibly due to arrhythmia. Postoperative cardiac catheterization performed in five patients showed reduction of the transmitral gradient from a mean of 16 mmHg to a mean of 5 mmHg. Calcification of the bioprosthetic valve occurred in two patients 3 1/2 years and 2 years respectively after the operation; one died from concomitant subaortic stenosis and one underwent conduit replacement. Although its long-term efficacy is limited, the SAV conduit seems the most reliable surgical option for infants and children with hypoplastic systemic atrioventricular valves unsuited to conventional surgery.

Repair of traumatic aortic arch to innominate vein fistula under deep hypothermia and circulatory arrest.

Penetrating injuries of the thoracic aorta are usually rapidly lethal. Few patients survive for long enough to undergo surgical treatment. When penetrating injuries of the thoracic aorta are complicated by arteriovenous fistula a correct preoperative diagnosis is important for adequate planning of the surgical repair, and so selective angiography is essential. The best approach is through a median sternotomy with the use of total cardiopulmonary bypass with or without deep hypothermia and circulatory arrest. Fistulae between aorta and innominate vein invariably lead to congestive cardiac failure. A review of the literature suggests that signs of cardiac failure rarely appear early. Congestive failure developed within 30 days of the initial trauma in only two of the 12 reported cases. In our case, the early onset of cardac failure refractory to therapy and the appearance of an expanding pulsatile mass at the base of the neck, threatening rupture, necessitated emergency surgical treatment.