Absent pulmonary valve syndrome: surgical treatment and considerations.

BACKGROUND: The operative management of absent pulmonary valve syndrome remains controversial regarding the need for pulmonary valve implantation and remodeling of pulmonary arteries. Moreover, symptomatic infants are considered to have a poor prognosis. This retrospective report summarizes the experience of a single institution. METHODS: Between May 1977 and May 1995, 37 consecutive patients underwent repair of absent pulmonary valve syndrome. Patients were divided into two groups according to age at operation: group A (10 infants less than 1 year old) and group B (27 patients older than 1 year). Mean age at operation was 5 +/- 4 months in group A and 72 +/- 42 months in group B. Initially, repair consisted of ventricular septal defect closure and relief of right ventricular outflow tract obstruction combined with pulmonary valve implantation. More recently, the concept of treatment has evolved with pulmonary arterioplasty without pulmonary valve insertion, except in patients with elevated pulmonary artery pressure. RESULTS: Of the 37 patients, 34 had successful repair. The overall in-hospital mortality rate was 8% (two deaths in group A and one in group B). No hemodynamic data were correlated with operative death. Death was associated with longer extracorporeal circulation time (p = 0.005) and longer aortic cross-clamping time (p = 0.019). In fact, these were clearly related to more complex anatomy (p = 0.001): multiple ventricular septal defects in 1, left pulmonary artery arising from the ductus in another, and left pulmonary artery arising from the aorta in the remainder. Follow-up was available in 22 of the 34 survivors. Mean follow-up time was 30 +/- 47 months in group A and 38 +/- 33 months in group B. All but 1 had no restriction of exercise, and most of them had pulmonary incompetence of Doppler echocardiography. One developed severe exercise intolerance because of pulmonary valve stenosis (xenograft), leading to uneventful reoperation 123 months after initial repair. One infant died suddenly of complete atrioventricular block 3 months after repair. The late mortality rate was 5%. CONCLUSIONS: Surgical treatment of absent pulmonary valve syndrome should include pulmonary arterioplasty to reduce bronchial obstruction, with no need for pulmonary valve insertion. This procedure is feasible and is recommended especially in markedly symptomatic infants.

Surgery for congenital mitral valve disease in the first year of life.

Between 1980 and 1993, 20 patients less than 1 year of age underwent operations for congenital mitral valve disease. Ten patients had congenital mitral incompetence and 10 had congenital mitral stenosis. Mean age was 6.6 +/- 3.4 months and mean weight was 5.6 +/- 1.5 kg. Atrioventricular canal defects, univentricular heart, class III/IV hypoplastic left heart syndrome, discordant atrioventricular and ventriculoarterial connections, and acquired mitral valve disease were excluded. Indications for operation were intractable heart failure or severe pulmonary hypertension, or both. Associated lesions, present in 90% of the patients, had been corrected by a previous operation in seven. In congenital mitral incompetence there was normal leaflet motion (n = 3), leaflet prolapse (n = 2), and restricted leaflet motion (n = 5). In congenital mitral stenosis anatomic abnormalities were parachute mitral valve (n = 4), typical mitral stenosis (n = 3), hammock mitral valve (n = 2), and supramitral ring (n = 1). Mitral valve repair was initially performed in 19 patients and valve replacement in one with hammock valve. Concurrent repair of associated lesions was performed in 12 patients. The operative mortality rate was zero. There were six early reoperations in five patients for mitral valve replacement (n = 4), a second repair (n = 1), and prosthetic valve thrombectomy (n = 1). One late death occurred 9 months after valve replacement. Late reoperations for mitral valve replacement (n = 2), aortic valve replacement (n = 1), mitral valve repair (n = 2), subaortic stenosis resection (n = 1), and second mitral valve replacement (n = 1) were performed in five patients. Actuarial freedom from reoperation is 58.0% +/- 11.3% (70% confidence limits 46.9% to 68.9%) at 7 years. After a mean follow-up of 67.6 +/- 42.8 months, 94% of living patients are in New York Heart Association class I. Doppler echocardiographic studies among the 13 patients with a native mitral valve show mitral incompetence of greater than moderate degree in one patient and no significant residual mitral stenosis. Overall, six patients have mitral prosthetic valves with a mean transprosthetic gradient of 6.2 +/- 3.7 mm Hg. These results show that surgical treatment for congenital mitral valve disease in the first year of life can be performed with low mortality. Valve repair is a realistic goal in about 70% of patients and possibly more with increased experience. Reoperation rate is still high and is related to complexity of mitral lesions and associated anomalies, but late functional results are encouraging.

[A journalist recounts]. / Un journaliste raconte.

Extraordinary and tragic story of Ernst Ferdinand Sauerbruch (1875-1951).

[Comparison of long-term results of arterial switch and Senning procedure in transposition of great vessels with intact ventricular septum]. / Comparison des résultats à long terme du switch artériel et de l'opération de Senning dans la transposition des gros vaisseaux à septum intact.

One hundred and five survivors after the 30th day of complete cure of transposition of the great arteries with intact ventricular septum between 1980 and 1985 were followed up. Fifty-four had an arterial switch (AS) in a single stage at an average age of 10 +/- 9 days and 51 had Senning's procedure (S) at an average age of 4 +/- 2.5 months. The average follow-up (97% of patients) was 5.8 +/- 1.1 years for the AS group and 9.3 +/- 2.3 years for the S group. The actuarial survival at 5 years was 100% in the AS group and 85.8% in the S group (p < 0.01) (8 late deaths). In the AS group, 3 patients were reoperated for stenosis of the pulmonary artery and, in the S group, 4 patients underwent 6 reoperations. All but 3 patients in the S group and all but 1 patient in the AS group are in functional Class I of the NYHA classification. Doppler echocardiographic studies have shown mild to severe dysfunction of the systemic ventricle in 2% of the AS group and 26% of the S group (p < 0.001). Holter monitoring, performed in 70% of patients in the S group showed sinus node dysfunction in 60% and sinus rhythm in 40% of cases. In conclusion, good functional results were observed at over 5 years in both groups. However, the absence of late mortality and the minimal incidence of systemic ventricular dysfunction in the AS group confirm the authors' choice of indication of arterial switch for the treatment of transposition of the great arteries with intact ventricular septum.

[Double-chamber right ventricle, aortic subvalvular stenosis and interventricular septal defect. Apropos of 12 cases]. / Ventricule droit à double chambre, sténose sous-valvulaire aortique et communication interventriculaire. A propos de 12 cas.

The authors report 12 cases of double-chamber right ventricle associated with discrete subaortic stenosis and ventricle septal defect. The statistics derived from 3,292 surgical reports of congenital heart diseases operated on at the Marie-Lannelongue Surgical Center over an 8 years period show that this association is 7 times more frequent than the law of chance. Twenty-two per cent of double-chamber right ventricles had an associated discrete subaortic stenosis and, in 9% of cases of subaortic stenosis a double-chamber right ventricle was observed. The cause of this malformation could be a developmental defect of the primitive interampullar ring.

[Immediate and mid-term results of surgery of aortic valve stenosis in the newborn infant]. / Résultats immédiats et à moyen terme de la chirurgie chez le nouveau-né avec sténose aortique.

Between January 1980 and June 1990, 47 consecutive neonates with severe aortic stenosis underwent surgical aortic commissurotomy at Marie-Lannelongue Hospital. The average age at operation was 5 days. Other cardiac abnormalities were present in 27 children and left ventricular fibroelastosis in 11 children. Closed heart aortic valvotomy via the apex of the left ventricle was performed in 26 patients and open heart commissurotomy in 21 patients. Immediate per- or postoperative death was observed in 14 cases (29.8%). Six patients died after open heart valvulotomy (29%) and 8 after closed heart valvulotomy (31%). The mortality was higher in children with a critical preoperative status: 60% versus 22% (p less than 0.05); in cases with an associated cardiac malformation: 45% versus 6% (p less than 0.01) or fibroelastosis: 91% versus 11% (p less than 0.001). None of the patients was lost to follow-up which lasted an average of 47 +/- 41 months. Ten secondary deaths were observed, of which 4 were sudden, 4 after reoperation and 2 due to mitral stenosis with pulmonary hypertension. The overall one year survival was 57% (IC 70%: 40-64); the 5 year survival was 46% (IC 70%; 30-60). Reoperation was necessary in 12 children. Survival without reoperation at 1 and 5 years was 49% (IC 70%: 41-56) and 43% (IC 70%: 29-59) respectively. At the last follow-up examination, 97% of the 23 survivors had good left ventricular function; the systolic pressure gradient was less than 50 mmHg in two thirds of patients and aortic regurgitation was minimal or absent in three quarters of patients. In critical aortic stenosis in neonates, surgery is associated with a high immediate and secondary mortality. The results at medium term in the survivors are good with respect to symptoms, left ventricular function and obstruction to left ventricular ejection.

Surgical management of isolated multiple ventricular septal defects. Logical approach in 130 cases.

From January 1980 through September 1990, 130 children underwent surgical closure of isolated multiple ventricular septal defects (mean age 14 +/- 18 months, mean weight 7.0 +/- 4.4 kg). Sixty-one were less than 1 year of age. Sixty-one children had pulmonary protection, 51 had pulmonary artery banding, and 10 had pulmonary valve stenosis. All other patients had severe pulmonary hypertension (mean systolic pressure 75.7 +/- 20.5 mm Hg and already disabling heart failure (New York Heart Association classes III and IV). The surgical management was based on the location of the defects and the ventricular dominance that were assessed preoperatively and intraoperatively. Midtrabecular ventricular septal defects were always centered by the moderator band and were therefore divided into low trabecular, midtrabecular, and high trabecular defects. The perimembranous septum was involved in 102 patients, the trabecular in 121, the inlet septum in 12, and the infundibular septum in 9. Fifty patients had the "Swiss cheese" form of the lesion. Closure of the ventricular septal defects included Dacron patch and mattress sutures. They were always first approached through a right atriotomy, which was sufficient for complete repair in 82 patients. In midtrabecular ventricular septal defects, section of the moderator band (n = 24) allowed closure of all the defects with a single Dacron patch. In 48 patients a right atriotomy and a right (n = 32) or left (n = 14) (particularly for low trabecular ventricular septal defects) or both right and left (n = 2) ventriculotomies were necessary to secure the repair. The hospital mortality rate was 7.7% (10 patients). The causes of deaths were residual ventricular septal defect (n = 5), pulmonary hypertension (n = 2), hypoplastic right ventricle (n = 1) and left ventricle (n = 1), and myocardial infarction (n = 1). Among eighteen survivors with residual ventricular septal defect, six were reoperated on; there were two deaths. A permanent pacemaker was necessary in four patients. Low trabecular ventricular septal defects and left ventriculotomy were significant risk factors for morbidity (death, residual ventricular septal defect), p less than 0.01. At 7 years of follow-up, 90% of survivors were in New York Heart Association class I. Actuarial survival and freedom from reoperation at 7 years were 89.6% and 87.5%, respectively.

[Development of a technique for the complete correction of transposition of great vessels]. / Evolution de la technique pour la correction parfaite de la transposition des gros vaisseaux.

Complete transposition of the great arteries is one of the most common cardiovascular anomalies. Several surgical methods of treatment have been proposed. Arterial repair theoretically seems a better option since it does not introduce any additional intra cardiac anomaly and it restores the left ventricle to its natival systemic function. The rationale for neonatal arterial repair lies on fetal and neonatal cardiopulmonary physiology. The left ventricle has to eject immediately after surgery a normal cardiac output at systemic pressure in the aorta. This is the case in the neonatal period, because during fetal life pulmonary artery and aortic pressure are equal. For simple TGA, after birth, with the fall in pulmonary vascular resistances and constriction of the ductus arteriosus, pulmonary artery and left ventricular pressures drop dramatically to less than one third (1/3) of systemic pressure. As a result, the left ventricle is not stimulated for growth adaptation and becomes a thin ventricle less contractile and more compliant. However, there is little doubt that during the first 2 to 4 post-natal weeks, the left ventricle is still suitable to sustain a systemic workload. Between april 1984 and april 1992, four hundred and twenty six (426) consecutive neonates underwent an arterial switch operation for various forms of transposition: with 34 hospital deaths. The mean age at operation was 13 days and the mean weight was 3.2 kg. Among patients with TGA-VSD and coarctation, 14 underwent a single stage repair through mid sternotomy. Actuarial survival rates were: 89% for TGA-IVS at 5 years, 90% for TGA-VSD, 85.3% for TGA-VSD and coarctation at 3 years.(ABSTRACT TRUNCATED AT 250 WORDS)

[Complete repair of tetralogy of Fallot in infants under the age of 6 months. Apropos of 25 cases]. / Cure complète de la tétralogie de Fallot chez le nourrisson de moins de 6 mois. A propos de 25 observations.

Between January 1982 and October 1988, 25 infants with Tetralogy of Fallot underwent total correction, total primary repair was carried out in 22 cases; 3 underwent correction after a palliative anastomosis. The average age was 3.7 +/- 1.6 months; the average weight was 5.06 +/- 1.41 kg and average body surface area was 0.30 +/- 0.06 m2. Nineteen patients had a regular anatomic form and 6 had an irregular form of the condition. The hospital mortality was 8% (2 cases): the mortality was nil in the regular anatomic form but 33% in the irregular anatomic forms. Twenty-one patients have been followed up for an average of 50.8 +/- 19.2 months. There were no late deaths; two patients were reoperated for a residual pulmonary stenosis; 19 patients are well and asymptomatic. Doppler echocardiography shows a residual pressure gradient between the right ventricule and pulmonary artery of 17.8 +/- 22.7 mmHg associated with a grade 1-2/4 pulmonary regurgitation. There are no residual ventricular septal defects or cases of atrioventricular block requiring permanent pacing.

Obstructed total anomalous pulmonary venous return. Toward neutralization of a major risk factor.

Among 57 neonates undergoing repair of total anomalous pulmonary venous return with severe pulmonary venous obstruction from 1980 through 1989, date of operation (1980 to 1984), preoperative hemodynamic instability, and failure to monitor pulmonary artery pressure postoperatively were risk factors for death. Thus, among the 30 patients having repair between 1985 and 1989, the 55-month survival rate including hospital deaths was 83%.

[Accessory mitral tissue responsible for left ventricular outflow obstruction. Reports of 7 cases]. / Tissu mitral accessoire responsable d'une gêne à l'éjection du ventricule gauche. A propos de 7 cas.

The authors report the medico-surgical experience of Marie Lannelongue hospital of a rare condition: accessory mitral valve tissue. Seven patients aged 2 to 28 years (average: 8.7 years) had left ventricular outflow obstruction due to accessory mitral valve tissue. The diagnostic was not obvious clinically and was based on the association of echocardiographic and angiographic data. This condition was associated with another intra-cardiac malformation in 6 of the 7 patients. Surgical treatment included resection of the accessory mitral valve tissue by an aortic or combined aorto-left atrial approach, together with correction of the associated intracardiac abnormality. The postoperative results were excellent with the regression of the ventriculo-aortic pressure gradient and the physiological integrity of the mitral valve.

Post cardiac surgery phrenic nerve palsy in pediatric patients.

From January 1978 to December 1988, 109 phrenic nerve paralyses (PNP) occurred in a total of 9149 cardiac operations performed in a population of patients younger than 15 years old (1.2%) whose age varied from 1 day to 15 years old and mean weight was 11.3 +/- 8.7 kg. PNP was diagnosed in 43 patients after closed procedures (1.2% of 3509 procedures) and in 66 patients after open heart operations (1.2% of 5640 operations). PNP was right sided in 49 cases and left sided in 60 cases. Open heart operations that predisposed to PNP were those which needed harvesting of autologous pericardium (P less than 0.0001) and wide exposure of the great vessels. The modified right Blalock-Taussig shunt was the main cause of PNP in closed procedures (P less than 0.02). Small children tolerated PNP less well. They needed longer ventilatory support (P less than 0.0005) and developed more respiratory complications. Seventeen children underwent plication of the affected hemidiaphragm and could be subsequently extubated. It is concluded that for prevention of PNP, a high level of attention should be exercised in neonates and small children, particularly when pericardium is harvested or when exposure needs extensive dissection of the great vessels and thymus resection, or at reoperation. We also prefer to avoid the use of iced slush lavage. PNP, when symptomatic, is best managed by continuous positive airway pressure (CPAP) ventilation. Diaphragmatic plication is recommended when after 2-3 weeks there is no recovery of diaphragmatic function or when there are troublesome respiratory complications.