Congenital or acquired atrioventricular block (AVB) is a rare disorder in the pediatric population, while celiac disease (CeD) is a common multisystemic autoimmune disorder that is characterized by intestinal manifestations as they are the typical clinical presentation. Sometimes CeD presents more complex multisystemic involvement which includes the heart. Cardiac involvement, such as dilated cardiomyopathy, myocarditis or conduction disease, have been mainly described in untreated adult patients with or without gastro-intestinal symptoms; rare cases of AVB and CeD have been also reported, particularly in association with extra-cardiac manifestations. We describe a case of a progressive acquired AVB block in a 4-year-old child, in which CeD was later diagnosed. A rapid and significantly improvement of the AVB grade has been obtained after the child started a strict gluten-free diet, and so we suggest including diagnostic exams for CeD in all of the children with acquired AVB.
Right ventricular outflow tract anomalies (RVOTAs), such as pulmonary stenosis (PS), pulmonary atresia (PA), and pulmonary insufficiency (PI), are typical cardiac anomalies in monochorionic twins, and they are complicated by twin-to-twin transfusion syndrome (TTTS). The aim of this study was to conduct a long-term postnatal cardiological evaluation of prenatal RVOTAs in monochorionic diamniotic twin pregnancies complicated by TTTS and treated with fetoscopic laser surgery (FLS) and to analyze possible prenatal predictors of congenital heart disease (CHD). Prenatal RVOTAs were retrospectively retrieved from all TTTS cases treated with FLS in our unit between 2009 and 2019. Twenty-eight prenatal cases of RVOTAs (16 PI, 10 PS, 2 PA) were observed out of 335 cases of TTTS. Four cases did not reach the postnatal period. CHD was present in 17 of the remaining 24 cases (70.8%), with 10 being severe (58.8%; 10/17); nine cases of PS required balloon valvuloplasty, and one case required biventricular non-compaction cardiomyopathy. The risk of major CHD increased with prenatal evidence of PS and decreased with the gestational age at the time of TTTS and with the prenatal normalization of blood flow across the pulmonary valve. Despite treatment with FLS, the majority of monochorionic diamniotic twin pregnancies complicated by TTTS with prenatal RVOTAs had CHD at long-term follow-up.
Fetal aortic valvuloplasty may be considered in critical aortic stenosis (AS), to prevent progression of AS to hypoplastic left heart syndrome (HLHS), which can be followed by fetal hydrops or death in utero. A multigravid woman was transferred at 29 +4 weeks of gestation, due to fetal cardiomegaly. The fetus was diagnosed with severe AS, cardiomegaly, a dilated LV with poor contractility, and mitral regurgitation. She was admitted to the hospital for fetal cardiac function monitoring. At 31weeks of gestation, fetal echocardiography revealed a thickened and dysplastic aortic valve with severe stenosis, accompanied by severe LV dilatation and dysfunction, as well as RV dilatation and dysfunction. A retrograde flow in the hypoplastic aortic arch, a left-to-right shunt across foramen ovale, and prominent a-wave reversal in the pulmonary vein Doppler were also found, suggesting evolving physiology of HLHS. Under ultrasound-guided approach, the fetal aortic balloon valvuloplasty was performed using biopsy needle through maternal abdominal wall, placenta, and fetal LV free wall, a guide wire, and a coronary balloon. After procedure complete, severe fetal bradycardia was developed. After intra-ventricular injection of epinephrine and atropine, the fetal heart rate was recovered. Fetal movement with biophysical profile was recovered within 12 hours after the procedure. At 35+3 weeks of gestation, fetal echocardiography showed a slightly improved cardiac function. A 3070 g female neonate was delivered vaginally at 38weeks of gestation with Apgar score 6/7 at 1/5 minutes. The neonate underwent operation including RV-PA conduit implantation for recurrent AS and LV dysfunction at 11months after birth. She is alive with tolerable condition.
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