Critical pulmonary stenosis in two successive siblings (original) (raw)
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Familial pulmonary valve stenosis, atrial septal defect, and unique electrocardiogram abnormalities
Journal of Medical Genetics, 1985
The familial association of pulmonary stenosis, atrial septal defect, and unique electrocardiographic abnormalities involving a mother and two children is reported. Familial pulmonary stenosis not occurring as part of a named syndrome or without associated multiple congenital abnormalities is rare. The constellation of pulmonary stenosis, atrial septal defect, and the particular electrocardiogram abnormalities present here is to our knowledge previously unreported. The pattern of inheritance is consistent with an autosomal dominant mode of transmission.
Pulmonary hypertension associated with congenital heart disease
2020
The spectrum of pulmonary hypertension associated with congenital heart disease (PH-CHD) is broad and complex. The most common aetiologies are related to left-to-right shunts, resulting in volume loading of the pulmonary circulation (precapillary pulmonary hypertension) and left-sided obstructive disease (post-capillary pulmonary hypertension). However, in addition to these basic classifications are patients with absent pulmonary arteries, large septal defects, duct-dependent lesions, cardiomyopathies, and, perhaps the most challenging, patients with single ventricle physiology lacking a subpulmonic ventricle. It is therefore imperative that physicians taking care of children with PH-CHD are well versed not only in the different physiologies present in these children, but also in those that result from their respective surgical palliations as well and how these flows and pressures relate to their pulmonary vascular disease burden. This chapter presents the case of a child with triso...
Pediatric Cardiology, 2018
We herein describe, for the first time, the fetal presentation of a case of ventricular inversion, restrictive ventricular septal defect, pulmonary stenosis, hypertensive left ventricle and double outlet right ventricle at 34 weeks of gestational age. Postnatal echocardiography confirmed the prenatal diagnosis. The patient was subsequently successfully palliated with a left ventricle to pulmonary artery conduit. This report illustrates the importance of detailed fetal echocardiography to ensure appropriate delivery and neonatal management, and to optimize outcome.
Pulmonary stenosis and atresia with intact ventricular septum during prenatal life
Ultrasound in Obstetrics and Gynecology, 2003
K E Y W O R D S: congenital heart disease; fetus; prenatal diagnosis; pulmonary atresia; pulmonary stenosis ABSTRACT Aim To identify fetal echocardiographic characteristics predictive of perinatal outcome in cases with a prenatal diagnosis of pulmonary stenosis or pulmonary atresia.
Genetic Perspective of the Congenital Heart Disease
Pakistan Heart Journal
Congenital heart diseases (CHDs) are the structural abnormalities that may occur in the heart, greater veins and arteries or may include the septum between the ventricles and atria that mainly happen during the developmental stages of the heart in the neonates. It is the most frequent birth defect affect 1% of all infants.1,2 The CHDs includes following major categories: septation defects, cyanotic heart diseases and left sided obstruction defects. The septation defects can be further divided into ventricular septal defects (VSDs), atrioventricular septal defects (AVSDs) and atrial septation defects (ASDs). The cyanotic heart disease occurs due to many defects such as tetralogy of Fallot (TOF), Ebstein’s anomaly of the tricuspid valve, double outlet right ventricle, pulmonary atresia, total anomalous pulmonary venous connection and tricuspid atresia. The left sided obstructive lesions involves the hypoplastic left heart syndrome, interrupted aortic arch, aortic stenosis, mitral sten...