A patient with polysplenia syndrome, dextrocardia, left atrial isomerism, normal great vessel relationships, and no intracardiac shunts developed progressive cyanosis and clubbing. Pulmonary arteriovenous malformations (PAVMs) were diagnosed by angiography and confirmed by lung biopsy. Superior mesenteric arteriogram revealed hypoplasia of the intrahepatic portal vein branches and a portosystemic shunt. The possible etiologies of PAVMs are discussed.
Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis
Malrotation of the bowel in patients with congenital heart disease associated with splenic anomalies
PULMONARY ARTERIOVENOUS ANEURYSMS AND FISTULAS. ANATOMICAL VARIATIONS, EMBRYOLOGY, AND CLASSIFICATION
Fulminant development of pulmonary arteriovenous fistulas in an infant after total cavopulmonary shunt
Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage
Additional pulmonary blood flow with the bidirectional Glenn anastomosis: does it make a difference?
Intrapulmonary arteriovenous shunting may be a universal phenomenon in patients with the superior cavopulmonary anastomosis: a radionuclide study
Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome
Congenital portosystemic venous connections and other abdominal venous abnormalities in patients with polysplenia and functionally univentricular heart disease: a case series and literature review
Augmentation of pulmonary blood flow with an axillary arteriovenous fistula after a cavopulmonary shunt
Diffuse pulmonary arteriovenous malformations (angiodysplasia) with unusual histologic features: case report and review of the literature
Rapid progression of intrapulmonary arteriovenous shunting in polysplenia syndrome associated with biliary atresia
Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease
Pediatric hepatopulmonary syndrome is seen with polysplenia/interrupted inferior vena cava and without cirrhosis
The biological "scrabble" of pulmonary arteriovenous malformations: considerations in the setting of cavopulmonary surgery
A 10-year-old boy with dyspnea and hypoxia: abernathy malformation masquerading as pulmonary arteriovenous fistula
A basic understanding of congenital extrahepatic portosystemic shunt: incidence, mechanism, complications, diagnosis, and treatment
Birth defects encompass structural and functional alterations that occur during embryonic or fetal development and are present since birth. The cause may be genetic, environmental or unknown and can result in physical and/or mental impairment. Here is the latest research on birth defects.