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Congenital Heart Disease in Pregnancy
Published in Afshan B. Hameed, Diana S. Wolfe, Cardio-Obstetrics, 2020
Double-outlet right ventricle (DORV) is defined as “a type of ventriculoarterial connection in which both great vessels arise either entirely or predominantly from the right ventricle” [13]. DORV accounts for 1% of all CHD [14] and is often associated with other anomalies such as coarctation, heterotaxy, and chromosomal mutations such as trisomy 13, trisomy 18, and chromosome 22q11 deletion. Surgical approach to DORV, and thus the associated residua and sequelae, depend on the relationship of the VSD to the great arteries, the size of the VSD, the size of the great arteries, size of the ventricles, and the atrioventricular valves. Ventricular-to-pulmonary artery conduits and baffles are typically used in two-ventricle repairs, and are associated with conduit stenosis or regurgitation, and baffle stenoses or leaks. Single-ventricle repairs are discussed below.
Congenital cardiac anomalies
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
From the list above, choose which one is the most likely syndrome associated with each clinical scenario below. Each option may be used once, more than once, or not at all. A 5-year-old boy with sleep apnoea, hypothyroidism, developmental delay and hypotonia, who at 4 months of age underwent closure of an atrial and a ventricular septal defect along with repair of the mitral valve.A 3-month-old infant with asplenia, three lobes in each of the right and left lungs, double-outlet right ventricle with complete atrioventricular canal defect and non-obstructed total anomalous pulmonary venous return.A 5-month-old girl with hypocalcaemia, immunodeficiency and tetralogy of Fallot.A 1-month-old boy with valvar pulmonary stenosis, atrial septal defect and hypertrophic cardiomyopathy, along with hypertelorism, short stature and cryptorchidism.A 4-day-old girl with a webbed neck, short stature, ovarian agenesis and hypoplastic left heart syndrome.
Tetralogy of Fallot
Published in Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček, Congenital Heart Disease in Adults, 2008
Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček
In the past, the classical criteria of TOF were: Right ventricular outflow tract obstruction (RVOTO) (infundibular stenosis, often in combination with valvular and supravalvular pulmonary artery stenosis).A large (nonrestrictive) perimembraneous VSD with extension to the outlet septum (malalignment VSD).Aortic overriding (<50%; at a higher degree of overriding, the defect is classified as a double-outlet right ventricle).Consecutive right ventricular hypertrophy.
Pulmonary artery banding in patients with functional single ventricle associated with pulmonary hypertension
Published in Clinical and Experimental Hypertension, 2021
Gang Li, Han Zhang, Xiangming Fan, Junwu Su
After the initial 97 PAB, 62 patients (65.3%) underwent the Glenn operation, 4 patients died before the Glenn operation. 1 patient with double outlet right ventricle and 1 patient with single ventricle and total anomalous pulmonary venous connection, who developed severe ventricular dysfunction, and suffered sudden cardiac death. 1 patient with tricuspid atresia and 1 patient with double outlet right ventricle, who developed severe pneumonia, and died of respiratory failure. There were 23 patients following the PAB with high pulmonary hypertension, and not suited to Glenn operation, that received targeted PH drug therapy. The targeted PH drug therapy include: bosentan at 15.6–31.25 mg/kg/day or sildenafil at 0.5–3 mg/kg/day. For reducing ventricular preload and controlling pulmonary circulation, Digoxin at 0.005 ~ 0.01 mg/kg/days and oral diuretic at 1 ~ 2 mg/kg/days was given once a day were added. During the follow-up, the pulmonary artery pressure decreased from (49.91 ± 10.24 mmHg) to (43.74 ± 9.68 mmHg). Mean follow-up duration after PAB was (3.5 ± 2.3) years.
Tetralogy of Fallot with isolated levocardia in a young female
Published in Journal of Community Hospital Internal Medicine Perspectives, 2019
Zeeshan Sattar, Hafez Muhammad Abdullah, Sohaib Roomi, Waqas Ullah, Adnan Khan, Ali Ghani, Asrar Ahmad
Preoperative echocardiography revealed the anatomy of levocardia, a large subpulmonary ventricular septal defect (VSD), and severe subvalvular pulmonic stenosis with a gradient of 80 mmHg. It also revealed atrioventricular (AV) discordance with right anterior aorta arising from morphologically left sided right ventricle and left posterior pulmonary artery arising more than 60% from morphologically left sided right ventricle. There was associated congenitally corrected transposition of the great arteries and a double outlet right ventricle (DORV). The morphologically left sided right ventricle was also hypertrophied. There was a single left sided superior vena cava and an inferior vena cava that drained into right atrium. The interatrial septum was intact. Other findings included a left aortic arch and an intact interatrial septum.
Prenatal Diagnosis and Management of Ectopia Cordis: Varied Presentation Spectrum
Published in Fetal and Pediatric Pathology, 2019
Gürcan Türkyilmaz, Sahin Avcı, Tugba Sıvrıkoz, Emircan Erturk, Umut Altunoglu, Sebnem Erol Turkyilmazlmaz, Ibrahim Halil Kalelioglu, Recep Has, Atil Yuksel
Although cardiac anomalies are seen in the majority of cases, they are difficult to detect in the first trimester. Cardiac defects are generally revealed in the second or third trimester due to adequate visualization of the fetus. Cardiac malformations are the constant feature of pentalogy of Cantrell, and various types have been described. Ventricular and septal defects are the most common intracardiac anomalies followed by tetralogy of Fallot [21]. Other cardiac malformations may include patent ductus arteriosus, hypoplastic left heart syndrome and univentricular heart with pulmonary stenosis. The detection of cardiac defects is crucial, especially in isolated cases, because the presence of cardiac anomalies exacerbates the prognosis [22]. Moreover, the presence of pericardial effusion on the antenatal US should raise suspicions of associated anterior diaphragmatic hernia and hence a meticulous search for the features of the pentalogy of Cantrell should be considered. Cardiac anomalies can be better evaluated with a combination of fetal magnetic resonance imaging (MRI) and echocardiography. It can be helpful for pediatricians and surgeons to determine prognosis and develop a plan regarding the possibility and timing of surgery [23]. In our series, we detected a double outlet right ventricle in a fetus at 16 weeks of gestation; also tetralogy of Fallot was observed in one fetus postnatally. We could not identify this anomaly prenatally probably due to the US technical difficulty in detailed fetal assessment in a triplet pregnancy.