[Management of aortic coarctation at the adult age]. / Prise en charge des coarctations aortiques à l'âge adulte.

Classical treatment of coarctation of the aorta consists of resection and suture through a left thoracotomy. However, over the last 20 years, balloon angioplasty, recently associated with stenting, has progressively supplanted surgery in the adult both in native forms and in recoarctions. Usually, the diameters of the balloon and stent are chosen to be the same as that of the aortic isthmus or proximal aortic arch without exceeding that of the aorta at the diaphragm. Moreover, the tendency now is to recommend stenting in cases of severe, tubular and long stenosis associated with proximal hypoplasia and in cases of residual gradients after dilatation. The complications of percutaneous techniques are the risk of restenosis (11-15%), aneurysm formation (5%), and a very small risk of dissection. However, it is recognised that stenting is associated with fewer complications than dilatation alone or surgery. After correction, the main problem is that of hypertension, often associated with persistence of a pressure gradient at the isthmus. Coarctation is often associated with a congenital bicuspid aortic valve in nearly 50% of cases and the valvular condition may progress to stenosis or incompetence requiring corrective surgery. In these cases, a dilatation of the aorta must also be suspected which may progress to an aneurysm. In addition, pregnancy is often complicated by maternal hypertension. The consequences are a high risk of abortion and, for the child, a prematurity, poor growth, and a small risk of recurrence of the cardiac disease. Pregnant women should be followed up in a multidisciplinary fashion and, when possible, problems of residual stenosis, aneurysm and hypertension should be controlled and corrected before the woman wishes to be pregnant. In practice, medium and long term follow up should be undertaken by specialist teams and comprise clinical examination, blood pressure investigations on effort and by ambulatory recording, Doppler ultrasonography of the aortic arch and aortic valve and MRI which has become the reference examination for the aortic arch. After the initial investigations, these tests should be repeated every 2 or 5 years in adults or sooner depending on the results of the initial work-up.

[Abnormal origin of the left coronary artery from the pulmonary artery: a retrospective study of 36 cases]. / Anomalie de naissance de la coronaire gauche à partir de l'artère pulmonaire: étude rétrospective de 36 patients.

The aim of this study was to show the evolution of diagnostic techniques revealing an abnormal origin of the left coronary artery and present the follow-up results of patients operated for this malformation at different ages. This retrospective study includes 36 children or adults, with a mean age at the moment of of 28 months old, ranging from 7 days to 39 years. In 9% of cases, patients were asymptomatic. Before 1990, 81% of children had a cardiac catheterization, versus 25% after. Indeed, echocardiography with color Doppler enabled the diagnosis of abnormal origin of the left coronary artery in 70% of cases. The pre-operative mortality is at 21% (12.5% after 1990). Twenty-three patients had surgery: left coronary artery reimplantation (n=16), bypass (n=5), at mean of 44 months old for the entire series, but 21 months old after 1990. The follow-up was clinical, ECG and radiographic and echocardiographic after 1975; when possible an exercise test, cardiac nuclear imaging and more recently coronary CT scan and MRI have been performed. In conclusion, children are operated earlier and the follow-up should focus on the detection of occlusion of the re-implanted left coronary artery by echocardiography, with or without stress, exercise test, cardiac nuclear imaging, MRI and sometimes, coronary angiography.

[Pulmonary embolism and right diaphragmatic paralysis: "one train can hide another"]. / Embolie pulmonaire et paralysie diaphragmatique droite "un train peut en cacher un autre".

The authors report the case of an 84 year old woman admitted for a mild pulmonary embolism associated with severe hypoxaemia. The association of a right diaphragmatic paralysis with renewed patency of a foramenovale and creation of a right-to-left shunt is probably an underestimated cause of refractory hypoxaemia.

[The Jervell and Lange-Nielsen syndrome. Natural history, molecular basis and clinical outcome]. / Syndrome de Jervell et Lange-Nielsen. Histoire naturelle, bases moléculaires et devenir clinique.

UNLABELLED: Data on the Jervell and Lange-Nielsen syndrome (JLN), the long QT syndrome (LQTS) variant associated with deafness and caused by homozygous or compound heterozygous mutations on the KCNQ1 or on the KCNE1 genes encoding the IKs current, are still largely based on case reports. We analyzed data from 186 JLN patients obtained from the literature (31%) and from individual physicians (69%). Most patients (86%) had cardiac events and 50% were symptomatic already by age 3. Their QTc was markedly prolonged (557 +/- 65 ms). Most of the arrhythmic events (95%) were triggered by emotions or exercise. Females are at lower risk for cardiac arrest and sudden death (CA/SD). A QTc>550 ms and history of syncope during the first year of life are independent predictors of subsequent CA/SD. Most mutations (90.5%) are on the KCNQ1 gene; mutations on the KCNE1 gene are associated with a more benign course. beta-blockers have only partial efficacy as 51% of the patients had events despite therapy and 29% had CA/SD. CONCLUSIONS: JLN syndrome is a most severe variant of LQTS, with a very early onset, major QTc prolongation, and is not well responsive to beta-blockers. Subgroups at relatively lower risk for CA/SD are identifiable and include females, patients with a QTc pound550 ms, without events in the first year of life, and with mutations on KCNE1. Early therapy with ICDs has to be considered.

[An experimental model of pulmonary valve implantation: a percutaneous and transventricular approach without cardiopulmonary bypass]. / Modèle de mise en place d'une valve pulmonaire: approche percutanée puis trans-ventriculaire sans circulation extracorporelle.

Pulmonary valve replacement by a catheter procedure remains a therapeutic challenge. In this report, the authors demonstrate the possibility of implantation of a porcine xenograft specially prepared on an auto-expanding stent (valved stent) in a sheep model. The porcine xenograft was prepared with hypotonic non-enzymatic solutions. It was sewn onto an auto-expanding stent (Luminex Bard) and inserted into an introduction sheath of 22-24 F (Gore) calibre. In a preliminary approach, the catheter was inserted through the jugular vein. Out of 6 attempts, it was possible to position the valved stent in the pulmonary position in two cases but all the animals died of different causes: tamponade, arrhythmias, air embolism. Following this experience, two valves were implanted through the superior and inferior vena cavae. This first percutaneous approach has been modified to a mixed medico-surgical approach with a transventricular introduction without cardiopulmonary bypass. This was performed through a left thoracotomy with puncture of the pulmonary infundibulum using the same systems of introduction and valved stent. Three implantations were successfully performed. In addition, a reduction of the size of the pulmonary artery was realised to prevent embolisation of the valved stent to the pulmonary artery or one of its branches. The transventricular approach is feasible for implantation of pulmonary valve prosthesis on a stent. This technique could be adapted for correction of pulmonary regurgitation after correction of Tetralogy of Fallot associated with reduction of the pulmonary infundibulum.

[Percutaneous angioplasty of native coarctation of the aorta. Results in children and young adults]. / Angioplastie percutanée de la coarctation native de l'aorte. Expérience d'un centre chez l'enfant et l'adulte jeune.

The authors report the experience of one centre in the treatment of native coarctation of the aorta by percutaneous angioplasty. Between 1999 and August 2005, 22 patients (7 girls, 15 boys) underwent dilatation of their coarctation by a balloon catheter at an average age of 11.8 +/- 5 years and body weight of 44 +/- 21 kg. In 20 patients, balloon angioplasty alone was performed and, in the other two, the angioplasty was associated with the insertion of a covered CP stent. The dilatation was performed with a balloon/coarctation ratio of 2.18 +/- 0.6 (1.7 to 3.5) and a balloon/isthmus ratio of 1.0 +/- 0.23 (0.7 to 1.87). After the procedure, ascending aortic pressure decreased from 134.7 +/- 23.4 mmHg to 125 +/- 22.7 mmHg (p= 0.0003); descending aortic pressure increased from 93.4 +/- 14.9 mmHg to 104.8 +/- 21.7 mmHg (p= 0.003); transisthmic pressure gradient decreased from 41.7 +/- 14.1 mmHg to 19.8 +/- 9.5 mmHg (p< 0.0001) and the diameter of the coarctation increased from 5.9 +/- 2.6 mm to 9.3 +/- 2.6mm (p= 0.0015). The two patients treated by covered CP stents had excellent immediate results without significant residual gradients. After dilatation (sometimes repeated, N= 13), a gradient > 20 mmHg persisted in ten patients (36% of cases) but no predictive factor was found on statistical analysis. There were no cases of acute aneurysm. In one patient, the femoral pulse decreased but returned to normal with heparin therapy. No patient required emergency surgical treatment after the angioplasty. During the clinical follow-up, echocardiography and MRI showed no signs of aneurysm and one patient had mild irregularity of the isthmic region. Three patients underwent surgery by resection suture for persistent coarctation (one combined with a Ross procedure); another patient was treated by a CP stent. In conclusion, percutaneous angioplasty of native coarctation of the aorta gives satisfactory results with few complications in bigger children and young adults. The results can be improved by using a slightly higher balloon/coarctation ratio.

[The effect of an endothelin receptor antagonist in Eisenmenger syndrome: a single-center experience of 11 patients]. / Effet d'un antagoniste des récepteurs de l'endothéline dans le syndrome d'Eisenmenger: expérience d'un centre à propos de 11 patients.

The Eisenmenger syndrome is a complication of congenital heart disease with significant left-to-right shunts, such as large ventricular septal defects, and corresponds to fixed pulmonary hypertension with shunt reversal. Bosentan, an inhibitor of endothelin A and B receptors, is a new molecule previously validated in the treatment of primary pulmonary hypertension. The authors report their monocentric experience of bosentan in 11 consecutive patients with the Eisenmenger syndrome treated for at least one year. This retrospective study comprised 7 females and 4 males with an average age of 34 years (range 17 to 51 years). The underlying lesion was ventricular septal defect (n = 4), atrial septal defect (n = 3), pulmonary atresia with septal defect (n = 4 of which 2 were treated palliatively). Before treatment, the patients were classified according to the NHYA functional class (I, II, IIIa and IIIb, IV or, respectively from 1 to 5) with a distribution in this series between Classes IIIa and IV (average 3.81 +/- 0.75) and from 3 to 10 on Borg's dyspnoea scale (average 6.54 +/- 2.29). The ambient oxygen saturation (SaO2) at rest was, on average 77 +/- 9%, the haemoglobin concentration 16.6 +/- 2.4 g/dl; hepatic transaminase levels were normal. The 6 minute walk test before treatment was 216 +/- 111 m with marked desaturation on exercise (49 +/- 18%). With Bosentan, patients were globally much better clinically with a decrease in dyspnoea, improvement in NHYA class and increased 6 minute walking perimeter. Improvement in NYHA class was observed from 3 months' treatment (3.0 +/- 0.8, p = 0.0002) and was sustained to one year (2.54 +/- 0.7, p< 0.001). An improvement of dyspnoea on Borg's scale was observed from the second month's treatment (5.56 +/- 1.65, p = 0.0201) and persisted throughout follow up to one year (3.81 +/- 1.32, p < 0.0001). Similarly, the 6 minute walking perimeter increased from the first control at 6 months (323 +/- 82 m, p < 0.0001) and at one year (322 +/- 62 m, p <0.0004). Finally, although a significant increase in SaO2 was observed at 6 months (p = 0.0032), this was hardly significant at one year (82 +/- 10 %, p = 0.0512). Transaminase levels did not rise significantly at the follow up visits (p = ns) and the haemoglobin concentration was unchanged. No patient died during the study period. This study showed a clear functional improvement in patients with Eisenmenger's syndrome treated with bosentan. The drug was well tolerated clinically with few adverse effects and a good safety margin of usage.

[Pulmonary atresia with an intact interventricular septum]. / Atrésie pulmonaire à septum interventriculaire intact. Expérience d'un centre à propos de 35 patients.

We report this centre's experience of the treatment and follow up of pulmonary atresia with an intact septum. 35 infants were seen during the neonatal period. Opening via catheterisation was attempted in 21 patients with initial success in 10, and 11 failures leading to urgent surgery (surgical opening, n=9) and isolated Blalock-Taussig anastomosis (n=2). Fourteen other patients underwent immediate surgery: surgical opening, n=3, and Blalock alone, n=11. Four patients died in the neonatal period: 1 after successful opening via catheterisation, 3 others after Blalock anastomosis. Five others with a Blalock anastomosis died suddenly later. By the end of follow up, 16 patients had undergone biventricular type repair, of which 7 required additional procedures. 10 others had undergone cavo-pulmonary type repair, including 4 infants in whom the initial strategy of biventricular repair had failed. The only predictive factors at birth for subsequent progression to biventricular type repair were: larger tricuspid diameter at echography (10.9 +/- 2.25 mm versus 6.34 +/- 1.74 mm, p = 0.0007) or at angiography (10.07 +/- 2.09 mm versus 8.04 +/- 2.42 mm, p = 0.039), and the right ventricular morphology (p = 0.0011) with more tripartite ventricles, and less bipartite or even unipartite ones in the biventricular group.

Echocardiographic follow-up after Ross procedure in 100 patients.

The Ross procedure could provide an ideal aortic valve replacement method in children and young adults. We evaluated midterm echocardiographic results to assess pulmonary homograft function as well as pulmonary autograft dimensions and function. In all, 105 patients (26 women and 79 men) underwent the Ross procedure; median age at implant was 29 years. All patients underwent free root replacement. Transvalvular gradients and autograft dimensions were measured at 3 levels (annulus, sinuses of Valsalva, and proximal aorta) at discharge, at 6 months, and annually thereafter. Perioperative mortality was 4.7%. The mean period for echocardiographic follow-up in 100 patients was 32.7 months (range 0.5 to 7 years), during which 4 noncardiac-related deaths occurred. Two patients underwent late reintervention. No moderate or severe regurgitation was recorded. There was 1 case of mild homograft regurgitation and 4 of mild autograft regurgitation at late follow-up. Autograft peak gradients were low and reproducible (5 +/- 2.8 mm Hg at discharge vs 5.5 +/- 3.5 mm Hg at last follow-up, p = NS). Homograft peak gradients increased significantly without severe obstruction (7.8 +/- 5.7 mm Hg at discharge vs 15.8 +/- 9.2 mm Hg at last follow-up). The diameter of the autograft annulus was stable during follow-up, whereas autograft dimensions at sinuses and proximal aorta increased significantly. One group of patients was identified with sinus diameter increases >20% (group A). The 90 remaining patients were classified into group B. The only parameter significantly different between the 2 groups was the sinus diameters measured at discharge (1.74 cm/m2 (group A) vs 1.92 cm/m2 (group B); p = 0.036). In 100 patients and with echocardiographic follow-up for up to 7 years, the Ross procedure showed excellent results. For 10% of patients, we observed a 20% dilation of sinus diameters, but in only 3 patients (3%) was this beyond the upper normal limit.

Postpneumonectomy interatrial right-to-left shunt: successful percutaneous treatment.

This report describes the case of a 67-year-old man in whom atrial right-to-left shunt developed after a right pneumonectomy, leading to dyspnea with severe arterial desaturation. Transcatheter occlusion of the patent foramen ovale was successfully performed using a buttoned device. Review of literature and mechanisms of these atrial right-to-left shunts are discussed.

Long-term cardiovascular consequences of undiagnosed intralobar pulmonary sequestration.

We report the case of a patient with a congenital aortic valve stenosis associated with a long-term undiagnosed intralobar pulmonary sequestration. The important blood flow through the aberrant artery led to progressive congestive heart failure and severe hemoptysis at the age of 25 years. We demonstrate the regression of cardiac symptoms and left ventricular diameter after surgical resection of the sequestration.

Technique to repair tetralogy of Fallot with absent pulmonary valve.

The syndrome of tetralogy of Fallot with absent pulmonary valve is characterized by aneurysmal dilatation of the pulmonary arteries causing tracheobronchial obstruction of varying degree. Relief of this obstruction is the main goal of the surgical repair and can best be achieved by appropriate pulmonary arterioplasty. We describe our current technique to repair this syndrome in infants and older children including pulmonary arterioplasty, ventricular septal defect closure, and right ventricular outflow tract reconstruction without valve insertion.

Effects of modified and classic Blalock-Taussig shunts on the pulmonary arterial tree.

BACKGROUND: The aim of this study was to assess by angiography the late effects of both classic and modified Blalock-Taussig shunts in terms of growth and development of stenosis and distortion. METHODS: At a mean of 51 months postoperatively, we retrospectively reviewed the results in 78 patients who underwent creation of Blalock-Taussig shunts (25 classic and 71 modified). RESULTS: At the level of the anastomosis, the shunt caused a reduction in diameter of the pulmonary artery in 49% of all shunts, major stenosis (>50% narrowing of the lumen) in 14%, and distortion of the pulmonary artery in 19%, findings that did not correlate with the type of shunt. Distortion did correlate with younger age at the time of shunt operation (p=0.01). CONCLUSIONS: After a Blalock-Taussig shunt, growth of the pulmonary arteries occurred but did not exceed the normal growth of the pulmonary arterial tree. Moreover, a shunt procedure can cause distortion and stenosis of the pulmonary artery, which may have important implications for future corrective surgical intervention. All these findings support earlier complete surgical repair of correctable congenital cardiac defects.

Aortic root replacement with a pulmonary autograft in young adults: medium-term results in 70 patients.

BACKGROUND: Pulmonary autograft aortic valve replacement has been introduced in our institution in selected adult patients in light of the known disadvantages and limitations of conventional prosthetic valves. METHODS: We prospectively evaluated the use of the pulmonary autograft in a series of 70 young adults (31.2+/-8.7 years, range 16 to 49 years) operated on from March 1992 to April 1997 with aortic root replacement only. RESULTS: There were no in-hospital deaths and two noncardiac-related late deaths during follow-up of up to 62 months (mean 33 months). Thromboembolic complications were not observed. One patient required reoperation for infective endocarditis 4.3 years after surgery. Discharge echo-Doppler studies showed normal autograft and allograft valve function. Serial echo-Doppler studies showed no significant progression of aortic insufficiency and no dilatation of the autograft. A severe stenosis of the pulmonary allograft developed in 1 patient. CONCLUSION: Aortic root replacement with a pulmonary autograft, although more complex than conventional prosthetic valve replacement, is a safe, effective, and reproducible procedure in properly selected adult patients. Long-term results remain to be evaluated.

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.

[Interventional catheterization in congenital heart disease in the adult]. / Cathétérisme interventionnel dans les cardiopathies congénitales de l'adulte.

Interventional catheterisation comprises performing a palliative or curative procedure during arterial or venous cardiac catheterisation. This procedure can be performed at any age, equally well in the newborn or adults affected by congenital cardiopathy: in order to definitively treat a congenital cardiopathy, unrecognised in infancy but diagnosed in adulthood, such as pulmonary valvular stenosis, coarctation of the aorta, persistent ductus arteriosus, ostium secundum type interatrial communication, coronaro-cardiac fistula, pulmonary arterio-venous fistula, and patent foramen ovale; in order to improve the haemodynamic state of a complex congenital cardiopathy, for example by creating an interatrial communication, or by opening the pulmonary route in a complex cardiopathy which cannot be completely repaired; in order to complete a surgical procedure, to treat recurrence of peripheral pulmonary stenosis or recurrence of coarctation, and to embolise the systemic vessels leading off the aorta or veno-venous anastomoses after cavopulmonary intervention. Paediatric cardiology can lead to occlusion of a left-right or right-left shunt with different devices, alleviation of an arterial or venous valvular stenosis with dilatation catheters, or implantation of endoprostheses in arterial or venous stenoses.

[Percutaneous implantation of endoprosthesis for stenosis of branches of the pulmonary artery. Apropos of a case]. / Implantation percutanée d'endoprothèses pour sténoses des branches de l'artère pulmonaire. A propos d'un cas.

The authors report the case of a young man operated for tetralogy of Fallot and in whom surgical treatment of severe stenosis of the origins of the right and left pulmonary arteries had failed. Correction of these stenoses was obtained by implanting percutaneously an endoprosthesis in the right pulmonary artery with a good result: control angiography showed that the stenosis had disappeared and an increase in the right pulmonary artery diameter from: 5 to 11 mm, and the left pulmonary artery diameter from 7 to 14 mm. Perfusion pulmonary scintigraphy showed equal flow in the two lungs.

[Calcification and healing of infectious myocardial pseudoaneurysms. Apropos of 2 cases]. / Calcification, mode de guérison des pseudo-anévrismes myocardiques infectieux. A propos de deux observations.

The authors present two cases of infectious myocardial pseudo-aneurysms: the first was secondary to an intracardiac foreign body with mitral valve endocarditis; the second to septicaemia of urinary origin. Both cases progressed to calcification of the pseudo-aneurysm which appeared to be a mode of healing. Reviewed 20 and 19 years respectively after the initial episode, both patients have normal cardiac appearances apart from the residual myocardial calcification.

[Scimitar syndrome complicated by stenosis of the right pulmonary vein. Apropos of 4 cases]. / Syndrome du cimeterre compliqué de sténose de la veine pulmonaire droite. A propos de 4 observations.

The authors report 4 cases of the scimitar syndrome with pulmonary hypertension by stenosis of an abnormally draining right pulmonary vein and they also review the literature. All cases were symptomatic from infancy. The diagnosis was confirmed by catheterisation which showed a significant pressure gradient between the right pulmonary vein and the inferior vena cava, and by angiography which demonstrated the stenosis. None of the treatments proposed (interventional catheterisation with dilatation and eventual implantation of a stent, surgery with treatment of the stenosis and reimplantation of the right pulmonary vein in the left atrium, or pneumonectomy) were satisfactory. However, it is possible that earlier treatment could be effective as changes in the pulmonary vascular bed seem to occur very early in these patients.

[Natural history and prognostic factors in primary endocardial fibroelastosis in infants]. / Histoire naturelle et facteurs pronostiques de la fibro-élastose primitive de l'endocarde du nourrisson.

Over a 33 year period, 127 patients under 2 years of age with dilated cardiomyopathies and appearances compatible with the diagnosis of primary endocardial fibroelastosis were admitted to the paediatric cardiac unit of the CHRU of Lille. The average follow-up was 8.9 +/- 6.7 years. Ninety-four children (74%) were cured, 16 (13%) had persistent cardiomegaly and/or left ventricular dysfunction on echocardiographic examination, and only 17 (13%) patients died (10 in the year following their initial hospital admission including 5 in the first week). The outcome of patients was not related to age at diagnosis, sex, cardiothoracic index, initial shortening fraction of the left ventricle or the period at which the patients were seen for the first time. On the other hand, the presence of a family history of cardiomyopathy was associated with a significantly worse prognosis. A recurrence of symptoms was the factor most closely correlated with a bad prognosis: 12 of the 19 patients (63%) with this evolution died, and 4 others (21%) had persistent myocardial dysfunction at the end of the study. These recurrences were often observed after premature withdrawal or after use of ineffective dosages of digitalis. In the authors' experience, dilated cardiomyopathy in neonates with clinical features of primary endocardial fibroelastosis is associated with a relatively high number of cures. Prolonged treatment with high doses of digitalis seems a determining prognostic factor.(ABSTRACT TRUNCATED AT 250 WORDS)