A case report of late physiologic repair of congenitally corrected transposition of the great arteries and pulmonary stenosis in a severely cyanotic patient: better late than never

Background Patients with congenitally corrected transposition of great arteries (ccTGA) not infrequently seek medical attention for the first time late in life. Optimal management of natural history ccTGA is debated and must be tailored. Case summary A 38 years old male patient was referred to our centre because of severe cyanosis and worsening dyspnoea. Investigations disclosed: situs solitus, mesocardia, double discordance, large ventricular septal defect (VSD), severe pulmonary stenosis, no significant atrioventricular valves regurgitation. The patient underwent physiologic repair: VSD closure, placement of a left ventricle to pulmonary artery conduit and epicardial atrio-biventricular pacemaker implantation. The conduit was intentionally undersized to promote tricuspid valve continence. Postoperative course was uneventful, trans-thoracic echocardiography showed good biventricular function without significant tricuspid regurgitation. At one month after discharge the patients is in NYHA class II. Discussion Management of late presenter patients with ccTGA depends on the associated lesion and estimation of surgical risk. In selected patients markedly symptomatic physiologic repair is a rationale option, providing a normal saturation and biventricular circulation with a significantly lower surgical risk as compared with anatomic repair.

Isolated ventricular inversion with hypoplastic right ventricle and pulmonary stenosis in an adolescent with situs solitus: a rare combination

Isolated ventricular inversion with situs solitus is a severe and rare congenital cardiac malformation characterised by an atrioventricular discordance but with ventriculo-arterial concordance. Here, we present the rare case of an adolescent with isolated ventricular inversion and hypoplasia of the left-sided morphological right ventricle and pulmonary stenosis, a first of its kind to be reported in the literature.

The Electrocardiogram in Nonsystemic Ventricular Pacing in a Patient with Congenitally Corrected Transposition of the Great Arteries and Dextrocardia

Congenitally corrected transposition of the great arteries (CCTGA) has a high incidence of dextrocardia and complete heart block. We describe a 34 year-old male with CCTGA, dextrocardia, and third degree atrioventricular block status-post dual chamber pacemaker that presented with acute heart failure syndrome. Cardiac CT Angiography confirmed CCTGA with situs solitus and dextrocardia. EKG with standard frontal leads showed a normal P wave axis and right-sided precordial leads showed a right bundle branch block pattern. With situs solitus there is a normal P wave axis on standard frontal leads. With nonsystemic ventricular pacing in CCTGA with dextrocardia left- and right-sided precordial leads show a left bundle branch block pattern and right bundle branch block pattern respectively. This case highlights electrocardiographic patterns seen with dextrocardia and morphologic reversal of ventricles. Subject Terms List: Dextrocardia, Electrocardiogram, Pacemaker, Transposition of the Great Arteries

Acute myocardial infarction in rare case of dextrocardia and situs solitus – recognizing the acute ischemic syndrome in the midst of the COVID-19 pandemic

The reduction of care for acute coronary syndrome during the COVID-19 pandemic is recognized. We describe a case of a patient with dextrocardia and situs solitus, a rare condition that occurs in adults in up to 1:900 thousand cases, admitted due to acute coronary syndrome in the context of paroxysmal atrial fibrillation. Some of the particularities of the congenital condition are described, such as epidemiology, associated structural abnormalities, and the strategy to perform the examination, as well as the discussion of anti-aggregation and anticoagulation therapies, given the concomitance with arrhythmia.

Heart–lung transplant in congenitally corrected transposition of the great arteries and dextrocardia patient

A 53-year-old male patient was presented to our institution with the clinical picture of biventricular failure. The echocardiogram revealed congenitally corrected transposition of the great arteries, dextrocardia with situs solitus, atrioventricular discordance and ventriculoatrial discordance, severe systemic and mitral valves regurgitation, and severe pulmonary hypertension (mean pulmonary artery pressure: 51 mm Hg). He underwent heart–lung transplant. He was discharged on postoperative day 25 with left ventricular ejection fraction of 60%–65%, and with oxygen independency.

Cardiac CT in criss-cross heart

A criss-cross heart is an uncommon congenital rotational anomaly. It accounts for less than 0.1% of all congenital heart defects. The anomaly is characterized by crossing of the atrioventricular connections caused by rotation of the heart about its long axis. It is commonly associated with diverse cardiac defects. Cardiac CT imaging of criss-cross heart is sparse. We present a case of 1-year-old child with chief complaints of bluish discoloration of the body and fast breathing. Cardiac CT revealed atrial situs solitus, criss-cross-atrioventricular connections, atrioventricular discordance, double outlet right ventricle and dextro-malposed great arteries (Van Praagh S,D,D).