Illustrated Imaging Essay on Congenital Heart Diseases: Multimodality Approach Part III: Cyanotic Heart Diseases and Complex Congenital Anomalies

Abstract

Evaluation of complex congenital heart disease (CHD) demands obtaining detailed and specific information regarding cardiac chambers, connections, muscle contractility and quantification of the flow across valves, septal defects and collaterals. Plain radiography remains starting point and mainstay as a global assessment tool in pre and postoperative evaluation of the patients. Echocardiography initiates detailed investigative process, guided by clinical questions. If additional information is needed [1][2][3][4], MRI provides most precise information required for preoperative and post-operative evaluation for CHD. MDCT is a valid alternative with some limitations (inadequate functional information, radiation dose and contrast related complications), essentially providing structural information necessary for surgical management. CT evaluation also provides details about pulmonary anomalies, structural information regarding lungs, secondary complications and evaluation of tracheo-bronchial compression. CT also provides detailed mapping of collateral vasculature, information about postoperative status of surgical procedures, grafts and bony changes in association with CHD [5][6][7][8]. Integrated multimodality approach is required for, the management of the complex CHD, often decided in consultation with a multidisciplinary team consisting of paediatric cardiologist, cardiac surgeon and an imaging specialist. Table/Fig -3a&b,4] is the most common congenital cyanotic heart disease, beyond the neonatal period, accounts for 6-10% [9]. Predominant pathology of TOF is the outflow narrowing of the right ventricle and varying degree of the narrowing of pulmonary artery and branches. Other components include ventricular septal defect and overriding of aorta. Plain radiography typically shows upturned cardiac apex due to right ventricular hypertrophy. (Coeur en Sabot cardiac configuration) Lungs show varying degree of oligemia reflecting the extent of pulmonary outflow obstruction. Prominent right aortic arch is noted in up to 25% of patients. Reconstitution of a pulmonary vascularity is achieved by systemic collaterals and left to right shunting at the ductal level. The goal of surgical procedures in TOF is to increase the capacity of the right ventricular outflow by surgical correction and increase pulmonary flow. Palliation in the form of systemic to pulmonary shunt is performed for increasing the capacity of pulmonary arteries in staged procedures.Imaging requirement is to demonstrate the extent and severity of narrowing of pulmonary outflow and arteries, demonstration of associated anomalies and showing extent of pulmonary atresia or discontinuity. Measurement of right ventricular volume is very critical for staged reconstruction of outflow. MRI is the procedure of choice for functional assessment although MDCT offers accurate anatomical information in this regard [10]. Evaluation of coronary artery anomaly, exclusion of large conus branch in the region of outflow and demonstration of anomalous right coronary from LAD...

Wnt11 regulates cardiac chamber development and disease during perinatal maturation

“…The altered anatomy of a CHD heart can be further complicated by the persistence of fetal shunt pathways, pathological flow patterns and hypoxemia (16)(17)(18). As such, the growing heart is particularly vulnerable to multi-factorial perinatal stresses.…”

Case Report on Tricuspid Atresia

“…The type of marriage of the parents is non-consanguineous marriage. All other members of the family were not having complaints about their health except for my patient who was being admitted to the hospital [16][17][18][19][20].…”

A Common Polymorphism in the MTHFD1 Gene Is a Modulator of Risk of Congenital Heart Disease

“…Although CHDs can vary from relatively mild to very severe, they can have a significant impact on pediatric morbidity and mortality [ 2 ]. CHDs are a very heterogeneous group of diseases, and several systems for their classification have been proposed, including according to symptoms (e.g., cyanotic and a-cyanotic) [ 3 , 4 ], etiology (e.g., the National Birth Defect Prevention Study [NBDPS]) [ 2 ], and the ICD-10 World Health Organization classification [ 5 ].…”