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.

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.

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.

Cor Triatriatum Sinistrum: A Rarest Aetiology of Cardiac Chamber Thrombi Mimicking Left Atrial Mass

Background. Cor triatriatum is a rare congenital cardiac anomaly, represent 0.1% of all congenital cardiac malformations and may be associated with other cardiac diseases in as many as 50% of cases. The natural history of this defect depends on the size of the communicating orifice between the upper and lower atrial chamber. Case Presentation. We reported case of cor triatriatum in a 12 years old girl with chief complaint of shortness of breath, middle chest discomfort and palpitation since 5 days prior admission. The diagnosis was based on clinical features, chest radiography, electrocardiography and transthoracic echocardiography. Chest radiograph showed rounded cardiac apex and double contour appearance. ECG showed sinus rhythm, 75 beat per minute, RAD, CCWR, RVH, RV strain pattern with ST depression and T-wave inversion in II, III, aVF, V1-V5. TTE revealed 2 chambers of left atrium, with restrictive supramitral membrane, dilated right atrium, right ventricle and left atrium, smallish left ventricle, proximal left atrial thrombus (5.96 x 3.44 cm), relative mitral stenosis, severe mitral regurgitation, mild aortic regurgitation and severe tricuspid regurgitation. A diagnosis of cor triatriatum sinister was made. The only treatment is surgical correction. Medical therapy, with ampicillin and heparin, was administered during admission. Conclusion. Cor triatriatum has been reported in a 12-year-old girl. The diagnosis is confirmed by clinical manifestations, chest radiography and echocardiography. The only therapy is surgical correction. From the field of cardiac surgeon, patients are advised to improve their general conditions before underwent surgical procedures.

Age of puppies at referral to veterinary cardiology specialists for murmur investigation

Background Cardiac auscultation is an important screening test at the first health examination of puppies because most clinically relevant congenital cardiac anomalies cause a loud murmur from birth. This retrospective study aimed to investigate the age at which dogs with suspected congenital cardiac anomalies were referred to a veterinary cardiology specialist for murmur investigation. A secondary aim was to establish the time interval between the visit to the cardiologist and the first available murmur documentation. The digital archive of a veterinary teaching hospital was searched for dogs with congenital cardiac anomalies and puppies with innocent murmurs during a 5-year period. Dogs had to be referred because of a murmur, and they had to undergo physical examination and echocardiography by a veterinary cardiology specialist. The health certificate section of the pet passport, and the medical records from the referring veterinarian, were reviewed to identify the date when the murmur was first documented. Results Of the 271 included dogs, 94% had a congenital cardiac anomaly and 6% had an innocent murmur. The dogs’ median age was 190 days when they were examined by the cardiologist. Only 10% of the dogs were referred by the breeder’s veterinarian, while 90% of the dogs were referred by the new owner’s veterinarian. The median age of the first available murmur documentation by a first opinion veterinary practitioner was 95 days. Conclusions Only 10% of the puppies in the present study were referred to a veterinary cardiology specialist for murmur investigation before they were sold to a new owner. Referral prior to re-homing would have been feasible if the murmur had been detected and documented by the breeder’s veterinarian, if referral was offered by the breeder’s veterinarian and the referral was accepted by the breeder.

Late-onset total anomalous pulmonary venous connection in a 70-year-old woman

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiac anomaly. There are a few reports of untreated TAPVC diagnosed in patients older than 60 years. Herein, we report the successful surgical treatment of TAPVC in a 70-year-old woman. A 70-year-old woman with TAPVC presented with symptoms of acute heart failure. We closed an atrial septal defect and performed tricuspid annuloplasty and commissurotomy of the pulmonary valve. Postoperative CT showed no residual shunt, and the pulmonary veins drained into the left atrium. She had an uneventful postoperative course. This report describes the case of the oldest known patient who underwent surgical treatment for TAPVC. Surviving into adulthood with little or no symptoms is uncommon in patients with TAPVC, and cases of late-onset TAPVC, such as our case, are rare. Nevertheless, close vigilance is necessary to prevent misdiagnosis in patients with this clinical presentation.