A Case of Osseocartilaginous Defects and Cardiovascular Anomalies: An Ambiguous Entity

A 24-year-old man presented with a history of palpitation and haemoptysis. He had a short stature, cardiac anomalies and physical deformities, including polydactyly, clubbing, cataracts and cyanosis. Echocardiography was performed, revealing both atrial and ventricular septal defects, along with severe pulmonary hypertension and Eisenmenger syndrome.

DMBT1 expression and neutrophil-to-lymphocyte ratio during necrotizing enterocolitis are influenced by impaired perfusion due to cardiac anomalies

Background Deleted in malignant brain tumors 1 (DMBT1) is involved in innate immunity and epithelial differentiation. It has been proven to play a role in various states of inflammation or hypoxia of fetal gastrointestinal and pulmonary diseases. Discrimination of pathogenesis in necrotizing enterocolitis (NEC) based on cardiac status improves the understanding of NEC in different patient subgroups. We aimed at examining DMBT1 expressions regarding their association with cardiac status leading to impaired intestinal perfusion, intraoperative bacteria proof, and a fulminant course of NEC. Methods Twenty-eight patients with NEC were treated surgically between 2010 and 2019 at our institution. DMBT1 expression was examined in intestinal sections using immunohistochemistry to detect DMBT1 protein. Associations of clinical parameters and DMBT1 expression were analyzed. Results We examined DMBT1 levels in 10 patients without cardiac defects and 18 patients with persisting ductus arteriosus (PDA) and congenital heart defects (CHD). Compared to patients without cardiac malformations, DMBT1 levels tended to score higher in patients with PDA/CHD (p = 0.2113) and were negatively correlated with C-reactive protein in these infants (p = 0.0172; r = − 0.5533). The number of DMBT1-expressing macrophages was elevated in the PDA/CHD-subgroup (p = 0.0399). Ratios of neutrophils and monocytes to lymphocytes were significantly higher in infants with PDA/CHD (p = 0.0319 and 0.0493). DMBT1 expression was significantly associated with positive bacterial culture of intraoperative swabs (p = 0.0252) and DMBT1 expression of the serosa was associated with a fulminant course of NEC (p = 0.0239). Conclusions This study demonstrates that DMBT1 expression may be influenced by cardiac anomalies with an impaired intestinal perfusion in the neonatal intestine. NEC in PDA/CHD infants is associated with more DMBT1-positive macrophages and a significantly elevated neutrophil-to-lymphocyte ratio.

Design of a Portable Electrocardiogram (ECG) for the Prevention of Cardiac Anomalies in Health Campaigns in Peru

Cardiac complications are caused by an unbalanced diet, the consumption of alcohol, smoking tobacco, in addition to the excess of polluting gases such as carbon dioxide (CO2), among other gases that are harmful to the human body, due to these types of problems, they arise more frequently in people, which lead to heart problems at an early age. An optimal prevention of these cardiac abnormalities is necessary using an Electrocardiogram (ECG), by using this equipment it is possible to detect the cardiac anomalies that the person may have, thus giving early prevention for cardiac abnormalities that may be occurring in the patient. The design of a low-cost ECG will be considered using the AD8232 module, which allows recording the electrical activities of the heart, obtaining an ECG using the Arduino, in addition the Bluetooth module will send the data collected from the ECG from a patient to a cellphone which will store this information, to be later analyzed by a cardiologist. This paper is intended for use in health campaigns, given that according to the Pan American health organization, 70% of deaths in the world are due to heart problems, due to this figure, it is expected to reduce the rate with early prevention, which consists of the implementation of this biomedical equipment in health campaigns that will have the basic functionalities for the analysis of the electrocardiogram and that is low cost compared to biomedical equipment currently that provide the same functionalities.

Diagnostic performance of 320 cardiac MDCT angiography in assessment of PDA either isolated or associated with duct dependent congenital heart disease

Background Patent ductus arteriosus (PDA) is one of most common congenital heart defects, it's a unique vascular structure that provides direct communication between pulmonary and systemic circulation. MDCT angiography is a good imaging modality for evaluation of the PDAs and detection of their exact morphological type; course and diameters, which is important before percutaneous closure or stenting procedure of the PDA, also for selection of closure hardware. The aim of this study was to assess the role of MDCT angiography in qualitative and quantitative evaluation of PDA and associated cardiac and\or extracardiac anomalies. Results Echocardiography detected PDA in 28\30 cases while cardiac MDCT detected PDA in all studied 30 cases confirmed by cardiac catheterization and/or operation. MDCT angiography had sensitivity 100% and specificity 100% for PDA detection. PDA originated from aortic isthmus in 15 cases, inferior surface of aortic arch in 11 cases and innominate artery in 4 cases. The most common morphological type of PDA was type A (cone\46.67%) followed by type C (tubular\23.3%), type D (complex\10%), type E (elongated\13.33%) and type B (window\6.67%). The spearman correlation coefficient test demonstrated poor correlation between size of aortic end and MPA (P = 0.75), and between size of pulmonary end and diameter of MPA (P = 0.99) and also demonstrated fair correlation between length of PDA and MPA (P = 0.018). PDA was isolated in 4\30 cases and associated with cardiac and\or extra cardiac anomalies in 26\30 cases included; ASD (n = 18), VSD (n = 16), pulmonary atresia (n = 7), transposition of great arteries (n = 5), teratology of Fallot (n = 4), aortic coarctation (n = 4), persistent truncus arteriosus (n = 3), tricuspid atresia (n = 3), anomalous of pulmonary venous return (n = 3), hypoplastic segment of aorta (n = 2), Ebstein's anomaly (n = 1), bicuspid aortic arch (n = 1) and left hypoplastic heart syndrome (n = 1). Conclusion Cardiac MDCT angiography was superior to Echocardiography in detection, quantitative and qualitative evaluation of PDA either isolated or associated with congenital cardiac and\or extracardiac anomalies and was superior to Echocardiography in detection of associated extracardiac anomalies rather than associated intra cardiac anomalies.

Sinus of Valsalva Rupture or VSD Shunt: Mystery Solved by Cardiac CT

Unruptured aneurysm of sinus of Valsalva is an asymptomatic pathology and diagnosed incidentally. This extremely rare anomaly can be associated with other congenital cardiac anomalies which can make the diagnosis and prognosis even more complex. We are reporting a case of a 12-year-old boy with progressive dyspnea and episodes of syncope. Multimodality imaging confirmed the diagnosis and paved the way for appropriate surgical treatment options.

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.

TOTALLY ANOMALOUS PULMONARY VENOUS CONNECTION OUTCOMES FROM A SINGLE CENTER

Objectives: There is limited data on totally anomalous pulmonary venous connection (TAPVC) repair results in our country. The aim of this study is to evaluate the early postoperative results of a large series of TAPVC patients operated in our clinic. Patients and Methods: The data of the patients who underwent TAPVC repair in our clinic between May 2005 and May 2021 were retrospectively reviewed using hospital records Results: A total of 150 TAPVC patients aged between 0 days and 39 years (median 3 months, IQR: 18 days – 9 months), 92 males (61%) and 58 females (39%) underwent surgical intervention. The median length of hospital stay was 12 (IQR:7-16) days. Overall mortality was 14% (21 patients). Seventeen out of 32 patients with additional cardiac anomalies (53%) and 4 out of 118 patients with isolated TAPVC (3%) died. The presence of additional cardiac anomalies was associated with mortality (p<0.00001). Mortality rate in univentricular patients was 59% (10 patients). This rate was higher in comparison to the mortality rate of biventricular patients (8%; p<0,00001). Three patients were reoperated due to postoperative pulmonary venous stenosis. Conclusion: The TAPVC outcomes in isolated and biventricular patients were favorable with a low mortality and postoperative pulmonary venous stenosis in this cohort. Patients with a single ventricle physiology and/or heterotaxia had a significantly increased risk of mortality, which might be due to the intrinsic challenges of the univentricular physiology regarding the balance between the pulmonary and systemic circulations. Tendency for increased mortality in the cases with preoperative pulmonary venous obstruction is a potential target for improvement.

Digital solution for follow-up in congenital cardiac surgery

Background: In this era of public scrutiny, there is an ongoing need for innovative methods for patient follow-up. Objectives: As part of a quality initiative, we developed an automated post-operative follow-up system for patients following discharge after cardiac surgery at Boston Children’s Hospital. Methods: Discharge Communication (DisCo) is a web-based system developed at Boston Children’s Hospital. An automated text and e-mail with a link to a health status survey are sent at 30 days and 1 year post-discharge in English/Spanish. If there is no response, surveys are completed via phone calls to the patient/patient’s physician or chart review. Responses are stored in the DisCo database and the patient’s medical record. Patients who underwent cardiac surgery and survived to hospital discharge from October, 2016 received the surveys. Results: Overall, 3345 30-day and 2563 1-year surveys were sent between October, 2016 and June, 2020. Of 3345 30-day surveys, there were 3191 responses (95%). Of 2563 1-year surveys, there were 1807 responses (71%). Most patients/families responded directly to the link at 30 days (65% for paediatrics/75% for adults) and at 1 year (72% for paediatrics/78% for adults). Multi-variable logistic regression revealed that higher complexity of cardiac lesion, presence of major non-cardiac anomalies and presence of major residua were associated with readmission and catheter/surgical reinterventions. Non-cardiac anomalies were associated with increased need for services for learning, development or behaviour. Conclusions: DisCo provides a successful web-based health status assessment of patients following congenital cardiac surgery. It helps to identify high-risk patients who need closer follow-up.

Neonatal infective endocarditis in a cardiac rhabdomyoma case: A rare presentation

Neonatal infective endocarditis (IE) is an exceedingly rare disease and usually not associated with cardiac rhabdomyomas or any underlying structural cardiac anomalies. Cardiac rhabdomyoma is also the most common benign primary cardiac tumor. The prognosis depends on the size, location of tumors, and outflow tract obstruction but can regress within 2 months of age and reduces the necessity of surgery. Due to the variable clinical features and course, we need to evaluate cardiac vegetation as soon as possible for better outcomes. A combination of these two conditions was not reported before. Here, we presented a case of IE with cardiac rhabdomyoma in a male baby which is first reported from Bangladesh previously.