Anatomic repair of corrected transposition or atrio-ventricular discordance: report of 8 cases.

BACKGROUND: It is well recognized that a right ventricle (RV) may eventually fail if working as systemic. This has led to arterial switch operation for transposition of the great arteries (TGA), but more recently to anatomic correction of 'corrected transposition' or atrio-ventricular discordance (AVD) combining an atrial switch and a ventricular outflow repair (arterial switch or Rastelli type). METHODS: We report here 8 cases of such anatomic correction of AVD in patients from 3 months to 10 years. Of these patients, 6 had situs solitus, and 2 situs inversus; 7 patients had a ventricular septal defect (VSD); 5 had an increased pulmonary flow, and 3 had a decreased pulmonary flow with pulmonary stenosis (PS) or pulmonary atresia (PA). In all, 6 had discordant and 1 had concordant ventriculo-arterial (VA) relations, and 1 had double-outlet RV, 2 had an associated coarctation, and 1 had a cor triatriatum. Of the 8 patients, 5 had pulmonary artery hypertension, 3 a moderate to severe tricuspid insufficiency and 6 had undergone one or more previous operations. Correction was done between 3 months and 10 years of age, with 7 having a Senning procedure (twice left-sided) and 1 a Mustard type operation. VA outflows were repaired with: 4 arterial switches, 1 left ventricle to aorta rerouting, 1 right ventricular outflow tract (RVOT) repair and 2 Rastelli. A tricuspid annuloplasty was done once. RESULTS: All patients survived the operation, but 2 patients needed a reoperation (both successful): late obstruction of pulmonary venous channel after a left-sided Senning, and residual VSD closure. Mean follow-up is 45 months (3-122). Of the 8 patients, 6 patients are in sinus rhythm, 2 have junctional rhythm. A patient with junctional rhythm and occasional tachycardia died suddenly 18 months after surgery 1 week after satisfactory evaluation. All have a normal left ventricle function as evidenced by echocardiography. CONCLUSIONS: Despite a more demanding procedure, the 'anatomic repair' of hearts with AVD is possible, even in infancy, with good early and mid-term result. Despite the potential late rhythmic problems of the Senning operation (somehow more frequent with AVD in our experience), it may become, with increasing experience, the procedure of choice in corrected transposition.

[Malignant quadrivalvular dysplasia of Marfan syndrome in a neonate]. / Dysplasie quadrivavulaire maligne de la maladie de Marfan à révélation néonatale.

The authors report the case of a neonate who died early of refractory heart failure and presented with all the characteristics of Marfan's syndrome in its early form with articular retractions. There was clinical evidence of mitral, tricuspid and major aortic regurgitation. Two-dimensional and doppler echocardiography confirmed the prolapse of highly dystrophic valves and the quadrivalvar regurgitation. Typical lesions of the connective-elastic tissue were found at autopsy. The occurrence of malignant Marfan's syndrome in neonates seems to be exceptional; the syndrome may take the rare "en contractures" form. Death is not always ascribable to the cardiac pathology, this pathology being often less generalized than in our case which seems to be the first one where such diffuse and massive valval lesions have been observed. This particular form was comparable to the "congenital polyvalvular disease" described by Bahrati and Lev, except that the skeletal and visceral abnormalities of Marfan's syndrome are absent in that disease.

[Intravenous sotalol in the acute treatment of supraventricular tachycardias]. / Le sotalol intraveineux dans le traitement aigu des tachycardies supraventriculaires.

The effects of intravenous sotalol (0.5 mg/kg in 6 minutes) were studied in 23 patients with supraventricular tachycardia (ventricular rate greater than 120 bpm) after failure of placebo (isotonic dextrose). Thirteen patients had atrial flutter or fibrillation and 10 a junctional tachycardia of recent onset. Sinus rhythm was restored in 4 of the patients with atrial flutter or fibrillation and the ventricular rate was slowed significantly in 3 patients (less than 100 bpm). In 3 other patients the ventricular rate decreased (31 to 35 p. 100) but remained above 100 bpm. Sinus rhythm was restored in 3 of the 10 patients with a junctional tachycardia five to twelve minutes after beginning the injection. A slight slowing of the heart rate (13 to 27 p. 100) was observed in the other 7 patients but the frequency remained over 100 bpm. Overall, a satisfactory result was obtained in 10 patients (43 p. 100) including 7 cases in which sinus rhythm was restored. Sotalol was well tolerated in 21 patients; one patient complained of cold in the legs and one patient developed asymptomatic bradycardia (46 bpm). This study shows that intravenous sotalol may be useful in the emergency treatment of supraventricular tachycardia.

[Sinus dysfunction associated with catecholaminergic ventricular tachycardia. Therapeutic implications]. / Dysfonction sinusale associée à une tachycardie ventriculaire catécholergique. Implication thérapeutique.

The presence of resting sinus bradycardia (less than or equal to 45 bpm) with junctional escapes in an 8 year old child with catecholamine induced ventricular tachycardia, raised the problem of an underlying sinus node dysfunction. This was an important consideration bearing in mind the potential risk of the high dose betablocker therapy aggravating the sinus bradycardia. Electrophysiological studies showed pathological sinus node recovery times. Betablocker therapy did not aggravate the bradycardia, even after 6 months' treatment. A review of the literature showed the association of sinus bradycardia and catecholamine-induced ventricular tachycardia to be relatively common (39 p. 100). However, no mention was found of aggravation of this sinus bradycardia by betablocker therapy.

[Causes of preoperative mortality in transposition of great vessels. 2 cases]. / Causes de mortalité préopératoire de la transposition des gros vaisseaux. A propos de 2 cas.

The prognosis of transposition of the great arteries improved tremendously with the development of an early medico-surgical strategy including balloon atrioseptostomy, prostaglandin infusion and the arterial switch operation within the first days of life. Nevertheless, some patients still die preoperatively. We report on two newborn infants whose fatal outcome was promoted by an inadequate intercirculatory mixing. Since the diagnosis was not immediately made, the restrictive foramen ovale resulted very quickly in deep metabolic acidosis and balloon atrioseptostomy performed yet in the first hours of life could not prevent death. We emphasize the importance of prenatal echographic detection of this defect, only way to plan a balloon septostomy immediately after delivery in those infants suffering from inadequate atrial mixing.

[Short and medium-term results of the Norwood intervention (first stage)]. / Résultats à court et moyen termes de l'intervention de Norwood (première étape).

We report the short and mid-term results of the Norwood procedure (Stage one) in 20 patients with hypoplastic left heart syndrome or univentricular heart with aortic obstruction. Seven patients were prenatally diagnosed. Preoperatively there was obstruction to pulmonary venous return in 6 cases, a mild to moderate tricuspid regurgitation in 6 cases, and 11 patients were supported by mechanical ventilation with multiorgan failure in 5 cases. The surgery was performed under cardiopulmonary bypass at a mean age and weight of 12.9 days and 3 kg, respectively. Nine patients (45%) died within 30 days postoperatively, whereas 5 had delayed sternal closure. The mean duration of mechanical ventilation and ICU stay were 5.7 and 11 days, respectively. Two patients were reoperated for bronchial compression and tracheotomy. Systemic venous thrombosis occurred in 5 patients. In multivariate analysis, an older age at surgery was correlated with postoperative hospital death (p = 0.03). Among the 11 patients discharged home after Stage one procedure, 5 patients underwent balloon dilation for recoarctation and one patient died at home. A bidirectional cavopulmonary anastomosis was performed in 8 patients at a mean age of 0.76 year, with one postoperative death. After a mean follow-up of 1 year (+/- 1.97 years), the 9 remaining patients are all in NYHA class I, at a mean age of 2.2 years. Their mean transcutaneous saturation is 81%. The Norwood procedure (Stage one) is associated with high hospital mortality. However, the functional status of the survivors is correct, like in patients with other type of univentricular hearts. Moreover, although the causes of death in our patients are often not clarified, other studies show that the leading causes of deaths in our patients are often not clarified, other studies show that the leading causes of death in those patients are correctable. In conclusion, the option of a Norwood procedure (Stage one) should be proposed in patients with hypoplastic left heart syndrome (or variant).

[Outcome of infants with hypoplastic left heart syndrome diagnosed in utero]. / Devenir des enfants atteints d'une hypoplasie du coeur gauche diagnostiquée in utero.

The authors report the results of prenatal diagnosis of the hypoplastic left heart syndrome since 1998 in the University Hospitals of Marseille and Montpellier. Twenty-four prenatal diagnoses of this condition were made in mothers with a mean age of 29 (18 to 40 years) and after a mean term of 22 (18.5 to 33) weeks of amenorrhea. Seventeen therapeutic abortions were carried out and 7 neonates born after a mean term of 39 (28 to 40) weeks, were admitted to the paediatric intensive care unit. Two patients required ventilatory assistance with one early death. The other patients were stable after surgery. A Norwood (first stage) procedure was carried out in 6 neonates at a mean age of 5 (1 to 6) days. There was only one survivor (17%). Prenatal diagnosis of the hypoplastic left heart syndrome allows cardiac and extracardiac evaluation of foetuses with this condition. Therapeutic abortions may be proposed and was the commonest choice of the parents in this study. On the other hand, despite better management of neonates with this prenatal diagnosis, the poor prognosis of the Norwood first stage procedure is unchanged. A systematic search for a restriction of the foramen ovale on foetal echocardiography could optimise neonatal management of this problem.

Accessibility of selected hospitals and medical procedures by means of aerial and transit network-based measures.

OBJECTIVE: This report investigates the use of linear and travelled route (transit network-based) distances in estimating the accessibility of hospitals to patients, for some selected hospital admission diagnostics. METHODS: For patients admitted to 14 public hospitals in Rio de Janeiro City, during 1996, under the ICD-9 headings "Complications of Pregnancy, Childbirth and the Puerperium" and "Disorders of the Circulatory System", average distances between the patient's district of residence and hospital of admission were calculated (both as Euclidean and as network-based distances). Data were obtained from the country's public health data processing agency. Geographic co-ordinates were obtained for districts of residence from the postal codes of the patients' residences. Distances were estimated with the TransCAD Geographical Information System, based on a map of the city transit network. There were 8654 patients admitted under the "Complications of Pregnancy" heading and 3439 under "Disorders of Circulatory System". RESULTS: Variations of up to a factor of 5.3, and up to 34 km, could be identified between linear and network estimates. CONCLUSION: While recognizing that network estimates have advantages, the literature on accessibility frequently argues that aerial estimates are a good approximation for those. The present results show that this is not necessarily the case.