A RARE CASE OF DEXTROCARDIA AND SITUS INVERSUS WITH TETRALOGY OF FALLOT IN A 23-YEAR-OLD FEMALE: A CASE REPORT

Tetralogy of Fallot (TOF) is commonly identified cyanotic congenital cardiac anomaly; however, its affiliation with dextrocardia and situs inversus is very rare. We describe a case of dextrocardia and situs inversus associated with TOF in a 23-year old girl who arrived with central shunt at the age of 17 years and later for total surgical correction with magnificent outcomes.

Maternal and Fetal Outcome of Pregnancy with COVID- 19 in A Tertiary Level Hospital, Bangladesh

Introduction: Pregnant women have long been recognized as a vulnerable population during infectious disease pandemics due to physiological changes in the immune, pulmonary, cardiac and coagulation systems. It is essential to acquire knowledge of pregnancy outcomes, potential complications and neonatal health conditions born to an infected mother with COVID-19. Material and methods: This cross-sectional observational study was conducted in Combined Military Hospital (CMH), Jashore from June 2020 to July 2021 among 100 hospitalized laboratory-confirmed COVID-19 positive pregnant women, patients who had clinical symptoms of COVID but RT PCR negative were excluded. The aim of the study was to evaluate the clinical profile and maternal and fetal outcome of pregnancy. Relevant data were recorded in a preformed data collection sheet and analyzed by SPSS version 20. Results: Among 100 COVID-19 positive hospitalized pregnant women, the mean age of participants was 27years (range 19-40 years), Maximum infection rate observed during 12 to 28 weeks of gestation among the participants, 21% got infected at 37 to 40 weeks of gestation and 20% got infected at 32 to 36 weeks. Seventy-four percent patients underwent delivery during the study & 23% of them continued with ongoing pregnancy; 67 of the participants underwent LUCS and 7 vaginal deliveries were done, 3% had abortion and IUFD 1% ,61% were multipara and 39% were Primipara, associated co-morbidities were subclinical hypothyroidism(15%), pregnancy induced HTN(12%) and GDM(8%); 36% participants were asymptomatic and 44% had mild symptoms, rate of LUCS was higher than (90.64%) vaginal delivery. Among the 73 live births, 80.82% were term and 10.18% were preterm of neonates, small for gestational was seen in the case of 20.55% neonates. Testing for SARS-CoV-2 was performed in all neonatal throat swabs and found positive in one case only. Eighty-six percent neonates were well-baby and 9.58% neonates required NICU admission. There were 2 neonatal deaths due to severe prematurity and 2 babies were found to have congenital cardiac anomaly and cleft lip, cleft palate. Though 36% of patients were asymptomatic but 10% were severe and in the critical stage. HDU support needed for 8% of patients and ICU support for 6%. Conclusion: This cross-sectional study supports that pregnant women with COVID-19 infection are at increased risk of adverse pregnancy and birth outcomes and a low risk of congenital transmission. Availability of ICU in critical conditions is needed for better pregnancy outcomes. J Bangladesh Coll Phys Surg 2022; 40: 10-16

Type A4 truncus arteriosus: series of 3 cases focused on dual source multidetector CT angiogram findings

Truncus arteriosus (TA) is a rare congenital cardiac anomaly caused by failure of normal conotruncal septation during the fetal development. This aberration leads to a common ventricular outflow artery over the malaligned large ventricular septal defect (VSD), supplying systemic, coronary and pulmonary circulation. People with such anomalous anatomy show variable presentation from early childhood to adult life depending on the severity of defects. We here present three cases of truncus arteriosus with aortic interruption / hypoplasia-coarctation (type A4 truncus arteriosus) with focus on relevant dual source MDCTA findings.

Prenatal diagnosis of isolation of aortic brachiocephalic artery

Background Isolated aortic brachiocephalic artery (IABA) is a rare congenital aortic arch anomaly. It is difficult to diagnose IABA prenatally and the prevalence in the prenatal population is unknown. Purpose To evaluate the echocardiographic characteristics and associations in fetuses with IABA. Material and Methods We retrospectively analyzed all cases of prenatal diagnosis of IABA from January 2012 to November 2020 and reviewed the follow-up results. Copy Number Variation Sequencing (CNV-Seq) was performed using the biological specimens of the of the fetuses and family members. Results Ten cases (10/45652, 0.022%) of IABA were identified in our center. The prevalence of the cases with isolated left subclavian artery (ILSCA) in the right aortic arch (RAA) population was 0.98% (6/613). The ILSCA was the most common isolated arch branch. All the isolated branches were on the opposite side of aortic arch in all the cases. The “ice stick” sign in the coronal section could be seen in most cases of IABA. Of the 10 cases, 8 (8/10, 80%) were associated with tetralogy of Fallot (TOF). Two cases of IABA were combined with 22q11.2 deletion syndrome. Conclusion IABA is a rare aortic anomaly. ILSCA was the most common isolated arch branch and TOF was the most common associated intra-cardiac anomaly. The “ice stick” sign in the coronal section could indicate a diagnosis of the IABA.

Fatal disseminated paradoxical embolism in inferior sinus venosus atrial septal defect

Inferior sinus venosus arterial septal defect (ASD) is a rare congenital cardiac anomaly. As with other types of ASDs and patent foramen ovale (PFO), this defect results in arteri-venous shunting with the risk of developing paradoxical embolism (PDE) to the systemic circulation from venous emboli. We report a case of a 67-year-old lady presented to emergency department (ED) with massive pulmonary embolism (PE) and recurrent acute limb ischaemia. On CT pulmonary angiography (CTPA), an incidental finding of inferior sinus venosus ASD was discovered that has caused the PDE. Thrombolysis was administered followed by open mechanical thrombectomy. The patient developed massive lower gastrointestinal bleed post thrombolysis then passed away despite embolization. This case report describes the catastrophic effect of PDE to cause disseminated multisystem thromboembolism. It also emphasizes the importance of early detection of a possible right-to-left shunt in patients presented with recurrent acute limb ischaemia. Transthoracic echocardiography has reduced sensitivity for inferior sinus venosus ASD requiring further assessment with transoesophageal echocardiography in selected cases.

The circle method for preoperative TAVI sizing in a Sievers type 0 stenotic bicuspid aortic valve

The most common congenital cardiac anomaly, affecting an estimated 0.4–2.25% of the general population, is the bicuspid aortic valve. The “pure” bicuspid aortic valve (non-raphe-type or bicuspid aortic valve type 0) is composed of 2 cusps, morphologically and functionally. The shape of the bicuspid aortic valve annulus is often elliptical, is relatively larger than the tricuspid aortic valves, and probably shows severe eccentric calcification. This situation contributes to the difficulties in selecting the correct type and size of transcatheter heart valve when treating bicuspid aortic valve stenosis. Furthermore, it is often associated with a dilated, horizontal ascending aorta and effaced sinuses. The goal of our video tutorial is to present the contemporary circle method used in preoperative sizing during TAVI procedures in patients with a bicuspid aortic valve as well as certain technical considerations and useful advice. Although annular sizing is the main focus for most patients with a bicuspid aortic valve, some patients may need the supra-annular level of sizing. For a dedicated sizing and positioning approach for the SAPIEN 3 Ultra valve, experts in the field propose the circle method.

Incidence of Heart Diseases in Children with Cleft Lip and Cleft Palate

Introduction: Cleft lip and cleft palate are one of the most common craniofacial malformations that occur in children. Congenital heart diseases are more prevalent among children with cleft lip and cleft palate. Early intervention is necessary to improve the survival and for proper development of children. Materials and Methods: A retrospective study is conducted at Paediatrics Department in Saveetha medical College for a period of one year. In the present study medical records of patients with cleft lip and palate are evaluated and analysed for the incidence of heart diseases. Results: In the present study medical records of patients with cleft lip and palate are evaluated and analysed for the incidence of heart diseases. Out of 163 children with cleft lip and cleft palate, 56 children (71.42% male and 28.57% female) had cleft lip, 30 children (66.66% male and 33.33% female) had cleft palate, 77 children (66.23% male and 33.76% female) had both cleft lip and cleft palate.VSD is reported as the most common cardiac anomaly (42.86%) in children with cleft lip and cleft palate. Conclusion: There is an increased incidence of congenital heart diseases in children with cleft lip and cleft palate. Echocardiography is an important investigation done for early identification and proper management.

Quadricuspid aortic valve and a large patent ductus arteriosus treated with device closure

Quadricuspid aortic valve is a remarkably rare congenital cardiac anomaly that predominantly becomes regurgitant with the passage of time. Aortic valve stenosis and aortic root dilatation are less common in quadricuspid aortic valve as compared to bicuspid aortic valve. The clinical presentation depends upon the functional status of the aortic valve, left ventricular function, and associated cardiac or coronary anomalies. The quadricuspid aortic valve is easily visualised during transthoracic echocardiogram with a characteristic X pattern of aortic valve in diastole. The association of quadricuspid aortic valve with patent ductus arteriosus is exceedingly rare. We are reporting a case of young girl with mildly regurgitant quadricuspid aortic valve, large patent ductus arteriosus, and volume-loaded left heart who underwent a successful device closure of her patent ductus arteriosus. To the best of our knowledge, such a case is being reported from Pakistan for the first time.

Cor Triatriatum Dexter: A Rare Cause of Aneurysmal Right Atrium

Cor triatriatum dexter (CTD) is a rare congenital cardiac anomaly with a diverse presentation in every age group. We report a case of CTD in a 36-year-old female who presented with palpitations due to giant right atrium (RA), which we managed successfully with surgical excision of the membrane and RA reduction.