An Adult Case of Polysplenia Syndrome Associated with Sinus Node Dysfunction, Dextrocardia, and Systemic Venous Anomalies

Heterotaxy Polysplenia Syndrome In An Adult With Unique Vascular Anomalies: Case Report With Review Of Literature.

The pattern of anatomical organization of the thoraco-abdominal visceral and vascular structures which is not the expected normal arrangement, is called as situs ambiguous or heterotaxy syndrome. Patients with heterotaxy syndrome exhibit a wide spectrum of anatomical variations involving thoraco-abdominal structures. We present here an incidental finding of heterotaxy syndrome associated with u...

Sinus node dysfunction concomitant with Brugada syndrome.

BACKGROUND A genetic correlation between Brugada syndrome (BS) and sinus node dysfunction (SND) has been proposed, although the clinical and electrophysiologic characteristics of this concomitant condition are unknown. METHODS AND RESULTS The study comprised 5 patients with symptomatic BS (4 with spontaneous episodes of ventricular fibrillation (VF) and 1 with syncope) of whom 3 had a documen...

Biliary Atresia Associated with Polysplenia Syndrome, Dextrocardia, Situs Inversus Totalis and Malrotation of Intestines

Biliary atresia (BA) is a rare disease and the end result of a destructive, inflammatory cholangiopathy, leading to fibrosis and biliary cirrhosis. It is classified into syndromic variety with various congenital anomalies and non-syndromic (isolated anomaly). We present here a 1-month-old female child with the syndromic variety of BA associated with polysplenia syndrome, dextrocardia, situs inv...

Drainage of left hepatic vein into coronary sinus associated with other systemic venous anomalies.

Coronary sinus aneurysm associated with multiple venous anomalies

BACKGROUND Congenital anomalies of the venous system are rare, involve the inferior vena cava (IVC), a persistent left superior vena cava (PLSVC), and the left hepatic vein (LHV), and can make cardiac diagnostic and therapeutic procedures difficult. CASE PRESENTATION We present a 67-year-old woman without heterotaxy syndrome associated with interruption of the left IVC that continued with the...