A new case of sodium-dependent multivitamin transporter defect occurring as a life-threatening condition responsive to early vitamin supplementation and literature review.

BACKGROUND: Biallelic pathogenic variants in SLC5A6 resulting in sodium-dependent multivitamin transporter (SMVT) defect have recently been described as a vitamin-responsive inborn error of metabolism mimicking biotinidase deficiency. To our knowledge, only 16 patients have been reported so far with various clinical phenotypes such as neuropathy and other neurologic impairments, gastro-intestinal dysfunction and failure to thrive, osteopenia, immunodeficiency, metabolic acidosis, hypoglycemia, and recently severe cardiac symptoms. METHODS: We describe a case report of a 5-month-old girl presenting two recurrent episodes of metabolic decompensation and massive cardiac failure in the course of an infectious disease. We compare clinical, biological, and genetic findings of this patient to previous literature collected from Pubmed database (keywords: Sodium-dependent multivitamin transporter (SMVT), SMVT defect/disorder/deficiency, SLC5A6 gene/mutation). RESULTS: We highlight the life-threatening presentation of this disease, the stagnation of psychomotor development, the severe and persistent hypogammaglobulinemia, and additionally, the successful clinical response on early vitamin supplementation (biotin 15 mg a day and pantothenic acid 100 mg a day). Metabolic assessment showed a persistent increase of urinary 3-hydroxyisovaleric acid (3-HIA) as previously reported in this disease in literature. CONCLUSION: SMVT deficiency is a vitamin-responsive inborn error of metabolism that can lead to a wide range of symptoms. Increased and isolated excretion of urinary 3-hydroxyisovaleric acid may suggest, in the absence of markedly reduced biotinidase activity, a SMVT deficiency. Prompt supplementation with high doses of biotin and pantothenic acid should be initiated while awaiting results of SLC5A6 sequencing as this condition may be life-threatening.

Use of spatiotemporal image correlation at 11-14 weeks' gestation.

OBJECTIVE: To assess prospectively the use of four-dimensional (4D) spatiotemporal image correlation (STIC) in the evaluation of the fetal heart at 11-14 weeks' gestation. METHODS: The study involved offline analysis of 4D-STIC volumes of the fetal heart acquired at 11-14 weeks' gestation in a population at high risk for congenital heart disease (CHD). Regression analysis was used to investigate the effect of gestational age, maternal body mass index, quality of the 4D-STIC volume, use of a transvaginal vs transabdominal probe and use of color Doppler ultrasonography on the ability to visualize separately different heart structures. The accuracy in diagnosing CHD based on early fetal echocardiography (EFE) using 4D-STIC vs conventional two-dimensional (2D) ultrasound was also evaluated. RESULTS: One hundred and thirty-nine fetuses with a total of 243 STIC volumes were included in this study. Regression analysis showed that the ability to visualize different heart structures was correlated with the quality of the acquired 4D-STIC volumes. Independently, the use of a transvaginal approach improved visualization of the four-chamber view, and the use of Doppler improved visualization of the outflow tracts, aortic arch and interventricular septum. Follow-up was available in 121 of the 139 fetuses, of which 27 had a confirmed CHD. A diagnosis based on EFE using 4D-STIC was possible in 130 (93.5%) of the 139 fetuses. Accuracy in diagnosing CHD using 4D-STIC was 88.7%, and the results of 45% of the cases were fully concordant with those of 2D ultrasound or the final follow-up diagnosis. EFE using 2D ultrasound was possible in all fetuses, and accuracy in diagnosing CHD was 94.2%. Five of the seven false-positive or false-negative cases were minor CHD. CONCLUSIONS: In fetuses at 11-14 weeks' gestation, the heart can be evaluated offline using 4D-STIC in a large number of cases, and this evaluation is more successful the higher the quality of the acquired volume. 2D ultrasound remains superior to 4D-STIC at 11-14 weeks, unless volumes of good to high quality can be obtained.

First trimester fetal echocardiography: where are we now?

The detailed study of the fetal cardiac anatomy in the first trimester of pregnancy by means of ultrasound is feasible whether using a transvaginal or a transabdominal approach. There is nowadays enough evidence that ultrasound in the first trimester of pregnancy is a safe procedure provided thermal and mechanical indices are taken into account. The best timing for successful imaging of the four chambers and great arteries in early gestation appears to be between around 13 to 14 weeks rather than 11 to 12 weeks. In experienced hands, first-trimester fetal echocardiography is quite sensitive for the detection of major structural cardiac abnormalities. Besides the nasal bone, markers for first trimester screening of chromosomal abnormalities such as nuchal translucency thickness, the flow in the ductus venosus and the flow through the tricuspid valve constitute also markers for cardiac abnormalities. The finding of an increased nuchal translucency, an abnormal flow in the ductus venosus or a tricuspid regurgitation constitutes an indication for more detailed fetal cardiac assessment. Other indication for a detailed cardiac assessment is the finding of an aberrant right subclavian artery and vascular anomalies. The emerging importance of these markers has caused renewed interest in the early study of the fetal heart.

Virtual autopsy by computed tomographic angiography of the fetal heart: a feasibility study.

OBJECTIVE: To determine the feasibility of postmortem computed tomographic (pm-CT) angiography for fetal heart evaluation. METHODS: Following termination of pregnancy (TOP) or intrauterine fetal death (IUFD) beyond 18 weeks' gestation, 33 fetuses were examined by pm-CT; in eight contrast medium was injected through the umbilical cord and in 25 contrast medium was injected directly into the heart. Logistic regression analysis was used to investigate the effect on the ability to visualize cardiac structures on pm-CT angiography of gestational age at TOP or delivery following IUFD, the time delay between fetal death and examination, the technique used for contrast-medium injection, the presence of cardiac abnormalities and whether or not there was IUFD. The diagnostic accuracy of pm-CT angiography for the evaluation of fetal cardiac structures was also evaluated. RESULTS: Cardiac anatomy including heart situs, the four-chamber view and great vessels could be visualized on pm-CT angiography in 29 out of 33 fetuses (87.9%). Logistic regression analysis showed that the ability to visualize cardiac structures on pm-CT angiography was positively correlated only with contrast medium injected directly into the heart. Twenty-five out of the 33 fetuses underwent conventional autopsy. There were five cases with suspected major cardiac abnormality at prenatal ultrasound and one with a minor cardiac abnormality. In one of these cases, severe leakage into the pleural cavity did not allow for visualization of any heart structure on pm-CT angiography and in another invasive autopsy was declined. In two of the remaining four cases, the findings on pm-CT angiography and invasive autopsy were in agreement, while in two a ventricular septal defect was found on invasive autopsy but not on pm-CT. None of the 27 cases with normal hearts was falsely classified as abnormal using pm-CT angiography. CONCLUSION: Pm-CT angiography by direct injection into the heart seems to be a feasible method for its evaluation. The extent to which such a technique could be used for the evaluation of congenital heart disease as an alternative to classical postmortem autopsy remains to be determined.

Magnetic resonance imaging in the normal fetal heart and in congenital heart disease.

OBJECTIVE: To evaluate prospectively the feasibility of magnetic resonance imaging (MRI) for assessment of the fetal heart for congenital heart disease (CHD). METHODS: This was a cross-sectional study, including 66 fetuses with a normal heart and 40 with CHD. The fetal heart was examined on MRI using axial steady-state free precession (SSFP) sequences. Regression analysis was used to investigate the effect on the ability to visualize cardiac anatomy of gestational age at examination, maternal body mass index, presence of fetal cardiac abnormality, fetal movements, fetal lie and twinning. The sensitivity and specificity of detecting cardiac defects were calculated. RESULTS: The four-chamber view was visualized in 98.1% of fetuses. The sensitivity of detecting a cardiac defect on the four-chamber view was 88% and the specificity 96%. The ability to visualize the left and right outflow tracts was only influenced by the presence of fetal movements: for the left outflow tract 94.4 vs. 50.0% visualization and for the right outflow tract 92.6 vs. 53.8% visualization without and with fetal movements, respectively. The sensitivity of detecting a cardiac defect of the left outflow tract was 63% and the specificity 100%, while sensitivity and specificity were 59 and 97%, respectively, for the right outflow tract. CONCLUSIONS: Despite the use of SSFP sequences, MRI in the fetal heart remains of limited value. It can only be used as a second-line approach for abnormalities of the four-chamber view suspected at prenatal ultrasound.

Use of a high-frequency linear transducer and MTI filtered color flow mapping in the assessment of fetal heart anatomy at the routine 11 to 13 + 6-week scan: a randomized trial.

OBJECTIVE: To prospectively assess the contribution of a high-frequency linear transducer and of moving target indicator (MTI) filtered color flow mapping in the visualization of cardiac fetal anatomy at the routine 11 to 13 + 6-week scan. METHODS: This was a cross-sectional prospective study, including 300 singleton fetuses at 11 to 13 + 6 weeks' gestation. Patients were randomized into four groups and a detailed fetal cardiac examination was conducted transabdominally using either a conventional curvilinear transducer, a conventional curvilinear transducer and MTI filtered color flow mapping, a high-frequency linear transducer or a high-frequency linear transducer and MTI filtered color flow mapping. Regression analysis was used to investigate the effect on the ability to visualize different cardiac structures of the following parameters: gestational age at ultrasound examination; fetal crown-rump length (CRL); maternal body mass index (BMI); transducer-heart distance; the technique used at ultrasound; and the position of the placenta. RESULTS: The four-chamber view was visualized in 89.0% of fetuses and regression analysis showed this rate was correlated with CRL and the use of MTI filtered color flow mapping during ultrasonography, and inversely correlated with BMI and transducer-heart distance. Use of a conventional curvilinear transducer and MTI filtered color flow mapping allowed visualization of the four-chamber view in 97.3% of fetuses, while this was only possible in 84.0% of fetuses using a high-frequency linear transducer. The left and right outflow tracts were visualized in 62.3 and 57.7% of fetuses, respectively. Regression analysis showed that the ability to visualize the left or the right outflow tract was correlated with the use of MTI filtered color flow mapping during scanning and was inversely correlated with transducer-heart distance. The use of a conventional curvilinear transducer and MTI filtered color flow mapping allowed visualization of the left and right outflow tracts in 96.0 and 97.3% of fetuses, respectively, while this was only possible in 37.3 and 26.7% of fetuses using a high-frequency linear transducer. CONCLUSIONS: During the routine 11 to 13 + 6-week scan, the use of MTI filtered color flow mapping but not of a high-frequency linear transducer, improves visualization of cardiac anatomy.

An unusual case of congenital cardiac cyst of the interventricular septum.

We present a very rare case of a neonatal multiple haemangiomatosis with a cystic interventricular cardiac haemangioma. An anechoic cyst of 10 mm of diameter, located in the superior third of the interventricular septum was found by foetal echocardiography at 30 weeks of pregnancy. The baby was born at 39 weeks of pregnancy and the skin was covered with multiple purple cutaneous haemangiomas. As neonatal haemangiomatosis may be associated with visceral haemangiomas, the diagnosis of cardiac haemangioma was considered and transthoracic echocardiography revealed a translucent cyst consistent with this diagnosis. At 6 months of age, the cardiac mass was barely visible and most cutaneous lesions had regressed without treatment. Cardiac haemangiomas must be considered in the diagnosis of cystic masses, and the present case emphasizes the tendency towards spontaneous regression of these lesions.

Prevalence of early postoperative arrhythmias in children with delayed open-heart surgery for severe congenital heart disease.

UNLABELLED: BACKGROUND; Our aim was to determine the incidence, risk factors and outcome of early postoperative arrhythmias in children with delayed treatment of severe congenital heart disease. METHODS: A prospective study was conducted in 141 consecutive children with delayed referral from emerging countries, who underwent open-heart surgery. RESULTS: Sinus node dysfunction was noted in 5 cases. Preoperative moderate extrasystoly was common and its incidence significantly increased in the postoperative phase. Overall, 9 patients required specific antiarrhythmic therapy: 6 for sustained atrioventricular reciprocating tachycardia, and 3 respectively for atrial flutter, atrial fibrillation and junctional ectopic tachycardia. Non-sustained atrioventricular and ventricular tachycardia required no therapy in respectively 6 and 1 case. Postoperative complete atrioventricular block was observed in 6 patients and remained permanent in 3. No major complications resulted from those arrhythmias. Preoperative low oxygen saturation, preoperative arrhythmias, as well as long cardiopulmonary bypass time and aortic cross-clamp time, were risk factors for early postoperative arrhythmias. CONCLUSIONS: Children with delayed surgery for congenital heart disease are at risk of developing early postoperative arrhythmias depending on the complexity of their disease and of its treatment. However, their prevalence (14%) is not higher than in the general population of cardiac children.

Management of cardiac emergencies in children.

Children with heart disease may present to the Emergency Department in many stages of life, with a range of cardiovascular manifestions, from minimally irritating palpitations to the life-threatening derangements of shock or lethal dysrhythmia. Cardiac emergencies are rare in children in comparison to adults. The pathophysiology differs: ischemic heart disease is virtually unknown, whereas most cases occur secondary to congenital heart disease. Their successful management requires an accurate diagnosis and timely interventions to achieve optimal outcomes in this heterogeneous and complex patient population. The diagnosis, however, is not always straightforward, as evidenced by the non-specific clinical picture that can be presented by pediatric heart diseases. This article reviews pertinent issues concerning diagnosis and management of cardiac disorders with which children present to the emergency department. The initial diagnostic and specific therapeutic approach to these patients will be discussed.

Spectrum and workload of consultation activities in a Belgian tertiary paediatric cardiac centre.

BACKGROUND: Few data are available on the spectrum and frequency of issues addressed in the paediatric cardiology consultation service of tertiary academic hospitals. METHODS: Those activities were collected prospectively during 6 months. RESULTS: A total of 967 consultations were performed. The origin was mostly the medical ward (n = 535), the intensive care unit (n = 195), the neonatal unit (n = 97), the operating room (n = 84) and the nursery (n = 44). In 553 cases, a heart disease was previously known and the most common reasons of consultation were postoperative (n = 279) and preoperative evaluations (n = 129). Thirteen newborns had a prenatal suspicion of congenital heart disease, which was confirmed in 11 cases. For the other 401 consultations, the most common clinical concerns included cardiac function in oncological disease (n = 60), murmur (n = 48), syncope (n = 33), diabetes mellitus (n = 28), prematurity (n = 27), syndrome (n = 19), unexplained stridor or respiratory distress (n = 19) and unexplained fever (n = 15). There were new diagnoses of congenital heart disease, acquired heart disease and arrhythmias in 35, 17 and 5 cases, respectively. CONCLUSIONS: The workload of the paediatric cardiology consultation service is increasing alarmingly. These data may be helpful in future planning of consultant manpower and in curriculum development in cardiac training of students and residents.

Delayed recognition of congenital heart disease.

OBJECTIVE: The aim of this prospective study was to evaluate the proportion of children with delayed recognition of congenital heart disease (CHD). METHODS: Of the 744 children with CHD primarily diagnosed during a 10 year period in one hospital, the patients were identified where the diagnosis of CHD was established with a significant delay. RESULTS: Sixty six patients (8.9%) had delayed diagnosis of CHD. Among patients with cyanotic CHD, 10.4% (7 of 67 cases) were referred after they had initially been discharged home from the birth clinic. Among patients with acyanotic CHD, 8.7% (59 of 677) of all children and 35.1% (59 of 168) of the children who required surgery or interventional catheterisation were referred at an age where elective repair should have already been performed or needed immediate treatment because of their haemodynamic status. Of the 66 patients with delayed diagnosis, one infant with cor triatriatum died at admission because of delayed referral and 10 children had severe complications: preoperative cardiogenic shock in seven cases of aortic coarctation and one case of endocardial fibroelastosis, pulmonary hypertensive crisis in one child after delayed repair of a ventricular septal defect, and infectious endocarditis after dental care in a teenager with undiagnosed moderate aortic stenosis, who required Ross operation a few months later. DISCUSSION AND CONCLUSION: A substantial proportion of CHD was detected with relevant delay. In all cases of late diagnosis, clinical cardiac findings were present that should have alerted the physician on the possible presence of underlying CHD.

[Diagnosis of congenital heart diseases]. / Diagnostic des cardiopathies congénitales.

Most of the relevant congenital heart defects are recognized by the neonatal clinical screening or even in utero by fetal echocardiographic screening. Nevertheless, a substantial percentage of defects are missed in the early screening and are diagnosed after discharge from hospital during childhood or even during adulthood. Often, this delay in making the correct diagnosis affects the overall outcome of the cardiac children. In almost all patients, cardiac findings are present that should have alerted the physician. This article reviews the importance of history and physical examination in the early diagnosis of congenital heart disease.

Association of a Cor Triatriatum Sinister and a right partial anomalous pulmonary venous return. A case report.

A 56-day old baby girl referred for recurrent respiratory tract infections and stridor, showed a rare association of a Cor Triatriatum Sinister (CTS) and a right Partial Anomalous Pulmonary Venous Return (PAPVR), in the form of a stenosed Scimitar vein, draining the right pulmonary veins to the subdiaphragmatic inferior vena cava (IVC), on echography and angiography. The baby underwent successful surgical repair of the CTS, by resection of the obstructive false membrane and reconnection of the Scimitar vein to the left atrium. At the age of 5 years, the child is doing well without any evidence of obstruction on the right or the left pulmonary venous drainage.

The use of dipyridamole to wean from inhaled nitric oxide in congenital diaphragmatic hernia.

A full-term neonate with a left-sided congenital diaphragmatic hernia (CDH) was ventilated mechanically by high-frequency oscillatory ventilation (HFOV). Despite inhaled nitric oxide (iNO) at a dose of 15 ppm, the neonate had severe respiratory acidosis and was placed on extracorporeal membrane oxygenation (ECMO) for 2 days. On day 7 of life, surgical repair of the CDH was performed. After the intervention, iNO (20 ppm) had to be restarted because of severe pulmonary hypertension (PHT). Ventilatory support and iNO then were weaned progressively. However, each daily attempt to discontinue iNO (from 2 ppm to 0 ppm), led to severe desaturation with significant right-to-left shunting. At the age of 33 days, dipyridamole (persantin) was administered intravenously at a dose of 0,4 mg/kg/min over 10 minutes and repeated every 12 hours for a total of 3 doses. After the second administration of dipyridamole, iNO could be stopped without rebound of PHT, and the neonate was extubated 1 week later. The authors report the use of dipyridamole for successful withdrawal of iNO. By inhibition of phosphodiesterase type 5, dipyridamole has the potential to increase the level of cyclic guanosine monophosphate in vascular smooth muscle cells, permitting vasodilation and restoration of endogenous NO. J Pediatr Surg 36:1864-1865.

Cavopulmonary connection after repair of pulmonary vein stenoses.

We report a case of heterotaxia with single-ventricle physiology undergoing successively pulmonary artery banding (PAB) at 8 months of age, repair of pulmonary vein (PV) stenoses at 15 months of age, and a fenestrated total cavopulmonary connection (TCPC) combined with repair of a regurgitant common atrioventricular (AV) valve and a Damus-Stansel operation for a restrictive subaortic ventricle at 5 years of age, followed by percutaneous closure of the fenestration. The child is now 10 years old and is, to the best of our knowledge, the only reported long-term survivor of a Fontan type procedure done after repair of PV stenoses.

Cardiac surgery with extracorporeal circulation in 23 infants weighing 2500 g or less: short and intermediate term outcome.

OBJECTIVE AND METHODS: From September 1990 to February 1997, 23 consecutive critically ill infants (12 males, 11 females) weighing 2500 g or less underwent cardiac surgery necessitating extracorporeal circulation (ECC). A retrospective study was carried out to evaluate short- and intermediate-term outcome. Mean weight at operation was 2265 g (range 1750-2500 g). Mean age at operation was 24 days. The indications for surgery were transposition of the great arteries (TGA; 7), ventricular septal defect (VSD; 4), aortic stenosis (AS; 3), univentricular heart (UVH; 2), tetralogy of Fallot (TOF; 2), interrupted aortic arch (IAA; 2), atrial septal defect (ASD; 1), atrioventicular septal defect (AVSD; 1) and total abnormal pulmonary venous return (TAPVR; 1). All patients were in NYHA class IV; 17 patients (74%) were intubated pre-operatively. RESULTS: The mean aortic cross-clamping time was 40 min. Twelve patients required deep hypothermia (<20 degrees C) with total circulatory arrest (mean duration 19 min). All patients were successfully weaned from extracorporeal circulation (ECC). Five patients left the operating room with an open sternum (mean duration before closure: 3.5 days). Mean duration of artificial ventilation was 10.6 days; of inotropic support 6.7 days and of intensive care stay 17.8 days. Severe complications were observed in 19 patients (83%): cardiac failure requiring high inotropic support (13), sepsis (7), and acute renal insufficiency (5). One patient needed a ventricular assist device. Five patients (22%) died in the intensive care unit (ICU): 2 AS with fibroelastosis, 2 IAA with VSD. and 1 UVH with pulmonary atresia. At discharge from the ICU, 7 patients were receiving no treatment. Mean duration of follow-up was 32 months (range 2-80 months). We had 2 reoperations: 1 for right ventricular outflow tract obstruction 1 year after a switch operation and 1 for mitral valve replacement 1 year after total abnormal pulmonary venous return repair (death 30 days post mitral valve replacement). Survival at I year was 73%. At the last clinical examination 16 patients were in NYHA class I. CONCLUSION: Despite the severity of pre-operative cardiac disease, early surgical repair with ECC in infants weighing 2500 g or less is feasible with tolerable mortality yet with significant early morbidity.

Nonvalved homografts of thoracic aorta in operation for complex congenital cardiac disease.

BACKGROUND: In contrast to the wide and successful use of valved aortic and pulmonary homografts, the non-valved prolongation of the thoracic aorta for the repair of some complex congenital heart diseases has rarely been described. We present here our experience with the use of descending aorta and aortic arch homografts as non-valved conduits for the surgical repair of complex cardiac malformations in 8 patients. METHODS: One atriopulmonary conduit replacement four extraatrial cavopulmonary connections, and one intraatrial cavopulmonary repair were achieved by means of a homograft of the descending aorta with a diameter of 15 to 17 mm. Three pulmonary unifocalization procedures were carried out in 2 more patients using a curved homograft of the aortic arch with a diameter of 18 mm. Except for 1 patient, whose incompetent common atrioventricular valve was replaced with a mechanical prosthesis, all other patients were managed without anticoagulation. RESULTS: No conduit-related complications were seen during a follow-up of 18 to 42 months. One patient died perioperatively during an emergency central repair of the right ventricular outflow tract after bilateral unifocalization with arch homografts. Another patient died suddenly 4 months after cavopulmonary connection. The remaining 6 patients are currently doing well. CONCLUSIONS: Regardless of the complexity of the underlying malformations, we are encouraged to use nonvalved thoracic aortic homografts in the repair of congenital cardiac diseases because of the reduction in thrombotic, hemorrhagic, and infectious complications associated with their use; convenience in handling; and their versatility in size and shape.

Echocardiographic and radionuclide pulmonary blood flow patterns after transcatheter closure of patent ductus arteriosus.

BACKGROUND: Transcatheter occlusion of patent ductus arteriosus (PDA) has been associated with protrusion of the occluder device into the left pulmonary artery (LPA). This study was conducted to evaluate the significance of occluder protrusion and its implications for potential obstruction of the LPA and associated decrease of left lung perfusion. METHODS AND RESULTS: Fifty-two patients underwent successful transcatheter PDA occlusion over a period of 5 years. In this study, 49 were reexamined between March and June 1995. In addition to clinical and echocardiographic examination, lung scintigraphy was performed. Protrusion into the LPA was present in 5 of 49 patients (10%). In these patients, maximal flow velocity in the LPA was significantly (P < .01) increased. Decreased left lung perfusion, defined as < 40% of total pulmonary blood flow, was found in 7 of 49 patients (14%). Although mean left pulmonary perfusion was significantly (P = .02) decreased in patients with protrusion, there was considerable overlap with patients without protrusion, and only a weak correlation was found (r = -.35, P = .01) between flow-velocity and left lung perfusion. CONCLUSIONS: Our results demonstrate that protrusion of the device in the LPA is an infrequent finding. If present, it is associated with increased maximal flow velocity in the LPA and diminished left lung perfusion. However, echocardiography and lung scintigraphy are weakly correlated: Increased maximal blood flow velocities in the proximal LPA proved to be a poor indicator or impaired left lung perfusion. Also, decreased perfusion occurs in the absence of echocardiographic evidence of device protrusion.

Insertion of an aortic and pulmonary homograft for simultaneous reconstruction of the left and right ventricular outflow tracts. A case report.

We present the case of the successful reconstruction in a child of a congenital cardiac malformation (tetralogy of Fallot) complicated by acquired aortic regurgitation and aneurysm formation of the left pulmonary artery due to previous endocarditis, by using an aortic homograft for reconstruction of the left ventricular outflow tract and a pulmonary homograft for reconstruction of the right ventricular outflow tract. Regarding the excellent results recently obtained with cryopreserved homografts, the many advantages of these valves compared to mechanical prostheses, we feel that aortic and or pulmonary homografts might constitute ideal biological valves for reconstruction of left and or right ventricular outflow tract in children when the presence of a congenital anomaly of the pulmonary valve renders an autograft impossible.

[Fetal cardiology: prenatal diagnosis of cardiac malformations]. / Cardiologie foetale: le diagnostic anténatal des malformations cardiaques.

Foetal cardiology is now a recognised chapter of both paediatric cardiology and foetal medicine. We describe here our experience with antenatal diagnosis of cardiac malformations and discuss both its genetic and epidemiological issues. Our experience confirms if need be that foetal medicine requires a well organised first-level foetal evaluation.