A Reassessment of the anatomical features of multiple ventricular septal defects

Over the course of time, new developments associated with embryogenesis of the murine heart have served to clarify the developmental processes observed in the human heart. This evidence allows for creation of a developmental framework for many congenital cardiac defects. Here, we aim to solidify the framework related to the categorization of both solitary and multiple ventricular septal defects. Mice having genetic perturbation of the Furin enzyme have demonstrated perimembranous and juxta-arterial ventricular septal defects, permitting the inference to be made that these defects can co-exist with defects occurring within the apical muscular septum. Based on developmental evidence, furthermore, all interventricular communications can be placed into one of three groups, namely, those which are perimembranous, juxta-arterial, and muscular. All of the defects are described based on their borders as seen from the morphologically right ventricle. Our focus here will be on those defects within the muscular ventricular septum, recognizing that such defects can co-exist with those that are perimembranous. We discuss the differentiation of multiple discrete defects from those referred to as the ‘Swiss cheese’ variant. As we show, appropriate surgical management requires understanding of the specific terminology, as the surgical approach may differ depending on the combination of the individual defects. Data from the Society for Thoracic Surgeons revealed that both mortality and morbidity were increased in the setting of multiple as opposed to solitary ventricular septal defects.

A newborn with Aortico-left Ventricular Tunnel mimicking Sinus of Valsalva Aneurysm

Aortico-left ventricular tunnel (ALVT) is a rare congenital cardiac anomaly and constitues of less than 0.1% of all congenital cardiac defects (1). ALVT is described as an abnormal connection between the ascending aorta and the left ventricul which originates commonly above the right sinus of valsalva. Most patients are diagnosed with an ALVT during early infancy (2). Although transthoracic echocardiography (TTE) is more effective in diagnosis of ALVT, misdiagnosis rate was 17.1% (3). Sinus of valsalva aneurysm (SVA) is frequently confused with ALVT (3). We report a term female newborn with SVA in echocardiographic examination but in surgery she was diagnosed with ALVT.

DOUBLE AORTIC ARCH - A CASE REPORT

Double Aortic Arch is a rare congenital cardiovascular anomaly. Its first successful surgery was performed by Robert Gross in 1945 at Children Hospital Boston, USA. It accounts for 0.4 to 1% of all congenital cardiac defects. Patients having a Double Aortic Arch mostly present with symptoms in the 1st week of life but depending upon the severity of symptoms can present at any age in childhood. We present a case report of two month old baby with noisy breathing, intermittent cough, gross jugular notch retraction and sub costal recession. His Cardiac CT was subsequently done which showed a Double Aortic Arch of left dominant variety encircling the trachea. Surgery was done and the encircling artery compressing the trachea was recognized, dissected and interrupted. Marked relief of tracheal and/or esophageal compression was evident from operation day. Post operative recovery was speedy. Patient’s follow up of was done at 1, 4 and 24 weeks. His recovery was unremarkable. Currently he was thriving well.

Pediatric congenital heart diseases: Patterns of presentation to the emergency department of a tertiary care hospital

Objective: To observe presentation of Pediatric congenital cardiac defects to the Emergency Department (ED) of a tertiary care hospital in Pakistan. Methods: This is a retrospective chart review of patients under the age of 16 years with congenital cardiac defects presenting to the Emergency Department of Aga Khan University Hospital over a period of eighteen months, from January 2012 to June 2013. Study population was divided into two groups; first group constituted children with undiagnosed congenital cardiac defects, whereas second group constituted children with diagnosed congenial cardiac defects presented to ED. In previously diagnose cases each visit was counted as a separate encounter. Results: Out of 133 children, 44 (33.5%) were diagnosed congenital cardiac disease for the first time (Group-1) in ED, while 89 (66.5%) children were diagnosed cases of congenital heart disease (Group-2). Among Group-1; main reasons for ED visits were cyanosis, cardiac failure, murmur evaluation and cardiogenic shock where as in Group-2; main presentations were cardiac failure, hyper cyanotic spells, gastroenteritis, lower respiratory tract infection, and post-operative issues. There were total 13 deaths. Conclusion: High index of suspicion is necessary for early diagnosis and management of children with congenital heart disease in the pediatric emergency department. doi: https://doi.org/10.12669/pjms.36.3.1592 How to cite this:Bano S, Akhtar S, Khan U. Pediatric congenital heart diseases: Patterns of presentation to the emergency department of a tertiary care hospital. Pak J Med Sci. 2020;36(3):---------. doi: https://doi.org/10.12669/pjms.36.3.1592 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.