Postnatal outcome following fetal aortic valvuloplasty for critical aortic stenosis.

OBJECTIVE: To report our experience of fetal aortic valvuloplasty (FAV) for critical aortic stenosis (AS), with a focus on the postnatal evolution of the patients. METHODS: This was a retrospective study including all fetuses with critical AS which underwent FAV in a single center between January 2011 and June 2022. FAV was performed under ultrasound guidance. Technical success was based upon balloon inflation across the aortic valve and improvement of the antegrade aortic flow across the aortic valve. At birth, a biventricular circulation (BVC) strategy was decided assuming the left ventricular (LV) systolic and diastolic function would ensure the systemic circulation. RESULTS: Sixty-three FAV procedures were performed in 58 fetuses, at a median (range) gestational age of 26.2 (20.3-32.2) weeks. The procedure was technically successful in 50/58 (86.2%) fetuses. There were 11/58 (19.0%) cases of in-utero demise and 9/58 (15.5%) terminations of pregnancy. No patient was liveborn after an unsuccessful procedure. Thirty-eight (65.5%) infants were liveborn, at a median (range) gestational age of 38.1 (29.0-40.6) weeks, of whom 21 (55.3%) required prostaglandin treatment. Twenty-eight of the 38 (73.7%) liveborn children (48.3% of the study population) entered the BVC pathway at birth. Among them, 20 (71.4%) required an aortic valvuloplasty procedure at birth (11 (55.0%) percutaneous balloon, nine (45.0%) surgical) and eight (28.6%) did not require any treatment at birth, but, of these, five (62.5%) underwent surgical valvuloplasty between day 26 and day 1200 of age. Eleven (39.3%) of the infants with BVC at birth required a second intervention and four (14.3%) of them required a third intervention. Two (7.1%) infants who entered the BVC pathway at birth underwent conversion to univentricular circulation (UVC). None of the surviving children with BVC developed pulmonary hypertension. The overall survival rate in those with BVC at birth was 22/28 (78.6%) at a median (range) follow-up of 23.3 (2.0-112.6) months. Ten of the 58 (17.2%) patients had UVC at birth. Among these, six (60.0%) received compassionate care from birth and four (40.0%) underwent surgery. Three of the 10 patients who were UVC at birth were still alive at the latest follow-up assessment, at a median (range) gestational age of 24.3 (8.3-48.7) months. CONCLUSIONS: FAV for critical AS led to increase of antegrade aortic flow in 86.2% of fetuses, with BVC being achieved in 48.3% (73.7% of the liveborn cases). Among patients with BVC at birth, the rate of reintervention was high, but 78.6% of these children were alive at the latest evaluation. © 2024 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Multifactorial origin of pulmonary hypertension in a child with congenital heart disease, Down syndrome, and BMPR-2 mutation.

A late preterm infant had pulmonary hypertension caused by a variety of mechanisms leading to complex management. This child had complete atrioventricular septal defect associated with mild left ventricular hypoplasia and Down syndrome diagnosed prenatally. The mother had been treated by antiretroviral HIV treatment during pregnancy. Aortic coarctation was diagnosed and rapidly repaired. After surgery, he required noninvasive ventilation for persisting elevated PCO2. Pulmonary CT scan showed normal bronchial tree, lung parenchymal abnormalities with mosaic aspect and hyperlucent zones, and indirect signs of lung hypoplasia with peripheral microbubbles. During follow-up, severe pulmonary hypertension was diagnosed on echocardiography without recoarctation, significant intracardiac shunting or diastolic dysfunction. The patient died after four months unable to be weaned from noninvasive ventilation. Post mortem lung biopsy showed abnormally muscularized arterioles with intimal fibrosis and pulmonary immaturity. Gentetic screening identified a BMPR-2 mutation. This patient illustrates the multifactorial origin of pulmonary hypertension in the neonatal period. The respective contribution of left-to-right shunt, post-capillary obstruction, and abnormally elevated pulmonary vascular resistances led to perform right heart catheterization to exclude excessive shunting and restrictive physiology of the left heart. Subjects with Down syndrome are also highly susceptible to decreased lung vascular and alveolar growth, which may increase the risk for pulmonary hypertension and lung hypoplasia. This case highlights two issues. The first one is that right heart catheterization should be discussed in neonates with unexplained pulmonary hypertension and the second is to extend indications of genetic testing for pulmonary hypertension genes in neonates who have unusual course of neonatal pulmonary hypertension, particularly in the setting of associated congenital heart disease (CHD).

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.

Transcatheter closure of secundum atrial defect in small children.

OBJECTIVE: To assess safety and efficacy of transcatheter atrial septal defect (ASD) closure in small children. BACKGROUND: Percutaneous closure of ASD is a well accepted alternative to surgery. Reported experience in small children remains, however, scarce. METHODS: Fifty-two children underwent percutaneous ASD closure at a weight < or =15 kg, in four Belgian tertiary referral paediatric cardiology centers. Indication for treatment, device implantation rate, procedural details, complication rate, residual shunt on echocardiography, and impact of procedure on symptoms were retrospectively analyzed. RESULTS: Mean age at procedure was 36 months (7-60), mean weight 13 kg (4.7-15). Associated cardiac lesions were seen in 21% of the patients and noncardiac in 32.7%. Most patients (69.2%) were symptomatic. Mean ASD size was 12 mm (range 5-20 mm). A device could be inserted in 49 patients (94%). No major complications occurred. Minor complications occurred in 8 patients (15.4%) including device embolization in 2, successfully treated with transcatheter retrieval and second device insertion. Mean follow-up reached 27 months (0.03-61.2). Residual shunt was absent, trivial, or small in 93% (n = 42) at latest follow-up. Clinical improvement was noted in 91.7% of the symptomatic patients. Minor complications were more frequent in the presence of large ASDs (>15 mm) but not in smaller babies (<10 kg). CONCLUSION: Patients < or =15 kg requiring ASD closure form a special population with high incidence of associated anomalies and medical conditions. For this particular set of patients, percutaneous closure is a valid alternative. Asymptomatic or mildly symptomatic children should be treated at a later age.

[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.