Jarcho-Levin syndrome, also known as spondylothoracic dysplasia and characterized by short trunk dwarfism, "crab-like" rib cage, with ribs and vertebral defects; it is not uncommon in Puerto Ricans. Many patients die in early infancy due to respiratory compromise associated to lung restriction and the reported cases emphasize mostly the skeletal malformations associated to the syndrome. We repo...

Isolated left ventricular (LV) apical hypoplasia is a rare congenital cardiac anomaly which is not accompanied by other cardiac abnormalities, with the exception of two cases. We report a case of a 33-year-old male patient with isolated LV apical hypoplasia combined with infundibular pulmonary stenosis and aortic stenosis. We review a literature focusing on the characteristic magnetic resonance...

Unilateral pulmonary artery agenesis with lung hypoplasia is very rare and only a few cases have been reported in the literature. Some patients with hypoplasia may suffer from recurrent hemoptysis dyspnea, and lung infections. Other patients are asymptomatic. Chest X-Ray reveal a reduction in the volume of hemithorax, an elevation in the diaphragm, reduced intercostals spaces and mediastinal sh...

Acute unilateral pulmonary edema is an unusual clinical condition occasionally associated with severe mitral valve insufficiency. We describe a patient diagnosed as having unilateral pulmonary edema after an acute anterior myocardial infarction. Echocardiograms performed in the acute phase ruled out mitral insufficiency. A perfusion lung scan showed left-sided pulmonary hypoperfusion. The diagn...

INTRODUCTION Isolated left ventricular apical hypoplasia is an unusual type of cardiomyopathy that presents with different clinical manifestations according to the age of the disease, ranging from no symptoms in children to congestive heart failure, pulmonary edema, or even malignant tachycardia in adults. To our knowledge, only a few cases of isolated left ventricular apical hypoplasia have be...

Persistent pulmonary hypertension of the newborn (PPHN) is a clinical disorder characterized by abnormal vascular structure, growth, and reactivity. Disruption of vascular growth during early postnatal lung development impairs alveolarization, and newborns with lung hypoplasia often have severe pulmonary hypertension. To determine whether pulmonary hypertension can directly impair vascular grow...

Congenital lung abnormalities are relatively rare but very dangerous for the fetus and neonate. This article describes the most common pulmonary complications observed during pregnancy such as congenital cystic adenomatoid malformation, diaphragmatic hernia, bronchopulmonary sequestration, hydrothorax, chylothorax, lung agenesia, pulmonary hypoplasia. The diagnostic methods and available therap...

Lung tissue developmental abnormalities are seldom reported. According to the classification of Schneider (1900), which was amended by Boyden (1955), they include pulmonary agenesis, aplasia and hypoplasia. Due to the early onset of symptoms, lung agenesis and aplasia are usually detected soon after birth. Conversely, lung or lobar hypoplasia may remain clinically silent for a long time. A sing...