A patient with atrial septal defect with cyanosis with diagnostic dilemma turned out to be a case of Total Anomalous Pulmonary Venous Connection (TAPVC)

Total anomalous pulmonary venous connection (TAPVC) is a congenital cyanotic heart disease where all 4 pulmonary veins do not open directly to left atrium. There are 4 types of TAPVC. Supra cardiac type forms a confluence and may open to Innominate vein or SVC. Cardiac type usually opens to coronary sinus. Infracardiac type opens to hepatic veins or other veins. Mixed type is the combination of others. Of these 4 types infracardiac type is most vulnerable and presents with early features of cyanosis, pulmonary hypertension, pulmonary vein obstruction. We present the case of an 18 year old lady with atrial septal defect (ASD) with cyanosis. Pre operative echo showed ASD with 3rd chamber behind LA, CT angiogram revealed large ASD, with tongue like extended chamber in posteromedial aspect of RA. Preoperative angiogram report was inconclusive. Despite the diagnostic dilemma, we took the challenge and the patient went for open heart surgery. The patient recovered well and discharged on 10 th post operative day. Post operative echocardiogram is encouraging and she is doing fine. KYAMC Journal.2021;12(02): 107-110

Epidemiology and Evaluation of Congenital Cyanotic Heart Disease in Children- A Review

Structure abnormalities of the heart or intrathoracic great vessels that arise during fetal development are known as congenital heart disease (CHD). It is the most frequent type of birth defect. Despite great success in surgical and medicinal care of CHD. many operations are palliative rather than curative. and some survivors still have significant residual hemodynamic and electrical conduction abnormalities. as well as long-term cardiovascular problems. The field of congenital heart defect treatment has come a long way in its infancy. With heart problems that were an automatic death sentence 60 years ago, to contemporary surgical survival rates of more than 96 percent for all defects evaluated together. No other branch of research or medicine has done so much in such a short time. In this paper, we overview epidemiology, evaluation and management of congenital cyanotic heart diseases in children.

Paraganglioma in an adolescent awaiting a cardiac transplant due to unrepaired congenital cyanotic heart disease and a univentricular heart: the first reported case

We report a case of a 16-year-old adolescent male born with univentricular congenital cyanotic heart disease (CCHD) who was diagnosed with an incidental paraganglioma while awaiting a cardiac transplant. The coexistence of paraganglioma and univentricular CCHD is very rare, with no previous cases described in the literature of a patient concurrently requiring a cardiac transplant. The complex physiology associated with a common atrium, common ventricle, aortopulmonary lung perfusion and a hypoplastic left lung rendered our patient extremely vulnerable to catecholamine-mediated effects of preload, contractility and afterload. The interactions and interdependence between these systems provided unique difficulties for perioperative management with serious implications for prospective cardiac transplant.

Assessment of bone mineral density in children with congenital cyanotic heart disease

Background: Cyanotic CHD is one of many disorders in paediatrics that influence the health of children in different clinical aspects. One of the fundamental aspects that may be affected is bone mineral density. Objectives: The aim of our study is to assess bone mineral density in children with congenital cyanotic heart disease of different anatomical diagnoses. Design/Methods: Cross-sectional, observational study included 39 patients (20 males) with congenital cyanotic heart disease of different anatomical diagnoses following with the cardiology clinic in Mansoura University children’s hospital. All patients were subjected to anthropometric measures, oxygen saturation assessment, and lumber bone mineral density using dual-energy X-ray absorptiometry. Results: Six patients (15.4%) out of the 39 included patients showed bone mineral density reduction, 13 patients (33.3%) showed bone mineral density with Z-score between −1 and −2, while 20 patients (51.3%) showed bone mineral density with Z-score more than −1. Conclusion: Low bone mineral density can be found in children with cyanotic CHD, making it important to consider bone mineral density assessment and early treatment if needed to avoid further complications.

A Rare Combination of Complex Congenital Heart Disease with Acquired Rheumatic Valvular Heart Disease

Tetralogy of Fallot (TOF) is a complex cyanotic congenital heart disease with a survival beyond middle age. Rheumatic and congenital cyanotic heart disease are common causes of hospital admission. However, coexistence of rheumatic heart disease with complex congenital heart disease like TOF is known to occur very rarely. This report presents a case of rheumatic valvular heart disease (AR & MR) with a complex congenital heart disease (TOF with PDA with PLSVC) in a 30 yrs old female. Patient was treated by conservative management and advised for corrective surgery and double valve replacement. Cardiovasc. j. 2021; 13(2): 235-238

Nephrotic Range of Proteinuria in Congenital Cyanotic Heart Disease: A Rare Complication

Nephrotic syndrome is a common renal problem in children. But it is an uncommon complication for cyanotic heart diseases. It has been rarely reported in pediatric population with congenital cyanotic heart disease in Bangladesh. We found two cases of congenital cyanotic heart disease those were referred to investigate for nephrotic range of proteinuria. Bangladesh J Child Health 2020; VOL 44 (3) :178-180

Cyanosis and Clipping: Noninfectious Aneurysm in a Patient with 22q11.2 Deletion Syndrome

Noninfectious cerebral aneurysms are rare in patients with congenital cyanotic heart disease. We present a patient with DiGeorge/velocardiofacial syndrome with a complex congenital cyanotic heart disease with a ruptured anterior communicating artery aneurysm. The 10-year-old child was managed by surgical clipping of the aneurysm. Surgical challenges included prominent veins in the Sylvian fissure, difficulty in differentiating arterial and venous bleed, and anesthetic risks. The patient recovered without any neurological deficits. This is the first report of a patient with 22q11.2 deletion syndrome, with a noninfectious cerebral aneurysm.

Three steps approach for preoperative evaluation of tetralogy of Fallot patients: role of 128 MDCT

Background Tetralogy of Fallot is the most common congenital cyanotic heart disease. Advances in surgical repair recently facilitate survival of the affected patients into adulthood with good quality of life. Despite imaging plays crucial role in diagnosis of TOF patients, no single diagnostic modality suffices for complete evaluation of TOF. Thus, different diagnostic tools should be used alone or in combination according to patient’s clinical question. This study aims to find out the agreement between the echocardiography and MDCT in evaluation of TOF patients prior to surgical repair. Results Three-step approach used in scanning TOF patients. Firstly, classic TOF findings, MDCT was comparable to echocardiography in overriding aorta (k = 0.6), VSD (k = 0.8), RVH (k = 0.4), and sub valvular pulmonary stenosis (k = 0.6). Echocardiography is superior to MDCT in evaluation of valvular stenosis (k = 0.4). MDCT was superior to echocardiography in supravalvular pulmonary stenosis. Secondary, surgical relevant findings, McGoon ratio calculated in 94.2%, small and major APCs in 45.5% and 28.5% respectively, PDA in 35% and anomalous coronary artery in 5.1%. Thirdly, other TOF-associated anomalies, common: ASD (34.3%) and RAA (28.3%), and persistent LSVC (10.3%). Uncommon: situs ambiguous (2.5 %), PAPVR (1.2%), and IVC anomalies (3.8%). MDCT was superior to echocardiography in surgical relevant findings and other TOF-associated anomalies. Conclusion MDCT is a crucial imaging tool for extra cardiac evaluation of TOF patients prior to surgical repair.