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Indian Journal of Critical Care... Nov 2020Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. We present a 61-year-old uncorrected TOF patient who presented with sepsis and...
UNLABELLED
Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. We present a 61-year-old uncorrected TOF patient who presented with sepsis and acute respiratory failure. At presentation, the patient had tachypnea, tachycardia, and oxygen saturation (SpO) 64%. Chest X-ray (CXR) showed bilateral basal opacities. 2D echo revealed left ventricular dysfunction, infundibulum stenosis, pulmonary atresia, overriding of the aorta, and ventricular septal defect. A provisional diagnosis of community-acquired pneumonia with septic shock with uncorrected TOF was made. He received empirical antibiotics, ventilatory support, and supportive care. Ventilator weaning was done accepting an arbitrary baseline SpO of 70-75% and lactate-guided volume resuscitation led to a successful outcome. Coronary angiogram showed collaterals. This case report illustrates the dilemmas faced in treating a critically ill uncorrected TOF. To the best of our knowledge, this case could be the oldest surviving uncorrected TOF patient in the Indian population.
HOW TO CITE THIS ARTICLE
Prakash SY, Kartik M, Rao M, Harde YR. Challenges Faced in Managing an Adult Uncorrected Tetralogy of Fallot Patient with Pneumonia and Septic Shock in the Intensive Care Unit. Indian J Crit Care Med 2020;24(11):1135-1136.
PubMed: 33384524
DOI: 10.5005/jp-journals-10071-23648 -
CJC Open Nov 2020Tetralogy of Fallot is a congenital heart disease comprised of a tetrad of ventricular septal defect, pulmonary stenosis, overriding aorta, and right ventricular...
Tetralogy of Fallot is a congenital heart disease comprised of a tetrad of ventricular septal defect, pulmonary stenosis, overriding aorta, and right ventricular hypertrophy. In developed countries, most cases are diagnosed in babies; mortality is high if not surgically corrected in a timely manner. We describe herein a woman who was diagnosed at age 73 years. Several factors accounted for her unusual longevity. We highlight the importance of multimodal imaging to look for other associated anomalies of tetralogy of Fallot in cases of apparent simple ventricular septal defect when the echocardiographic images are either suggestive or suboptimal.
PubMed: 33305230
DOI: 10.1016/j.cjco.2020.06.011 -
Frontiers in Pediatrics 2020The pathognomonic feature of tetralogy of Fallot (ToF) is the antero-cephalad deviation of the outlet septum in combination with an abnormal arrangement of the...
The pathognomonic feature of tetralogy of Fallot (ToF) is the antero-cephalad deviation of the outlet septum in combination with an abnormal arrangement of the septoparietal trabeculations. The aim of this article was to study perinatal hearts using Polarized Light Imaging (PLI) in order to investigate the deep alignment of cardiomyocytes that bond the different components of the ventricular outflow tracts both together and to the rest of the ventricular mass, thus furthering the classic description of ToF. 10 perinatal hearts with ToF and 10 perinatal hearts with no detectable cardiac anomalies (control) were studied using PLI. The orientation of the myocardial cells was extracted and studied at high resolution. Virtual dissections in multiple section planes were used to explore each ventricular structure. Contrary to the specimens of the control group, for all ToF specimens studied, the deep latitudinal alignment of the cardiomyocytes bonds together the left part of the Outlet septum (OS) S to the anterior wall of the left ventricle. In addition, the right end of the muscular OS bonds directly on the right ventricular wall (RVW) superior to the attachment of the ventriculo infundibular fold (VIF). Thus, the OS is a bridge between the lateral RVW and the anterior left ventricular wall. The VIF, RVW, and OS define an "inverted U" that roofs the cone between the interventricular communication and the overriding aorta. The opening angle and the length of the branches of this "inverted U" depend however on three components: the size of the OS, the size of the VIF, and the distance between the points of insertion of the OS and VIF into the RVW. The variation of these three components accounts for a significant part of the diversity observed in the anatomical presentations of ToF in the perinatal period.
PubMed: 33072668
DOI: 10.3389/fped.2020.503054 -
Journal of Investigative Medicine High... 2020Tetralogy of Fallot is the most common cyanotic congenital heart defect consisting of an overriding aorta, right ventricular outflow obstruction, ventricular septal...
Tetralogy of Fallot is the most common cyanotic congenital heart defect consisting of an overriding aorta, right ventricular outflow obstruction, ventricular septal defect, and right ventricular hypertrophy. Without surgical management, approximately only 3% of patients survive past the age of 40 years. Cases of unoperated patients reaching adulthood have been reported; however, few studies describe treatment guidelines for surgical or therapeutic management. In this article, we report the case of a 59-year-old Hispanic male with unoperated tetralogy of Fallot presenting to our cardiology clinic for initial workup and management.
Topics: Anticoagulants; Atrial Fibrillation; Cardiac Catheterization; Disease Management; Eisenmenger Complex; Electrocardiography; Humans; Hypertension, Pulmonary; Hypertrophy, Right Ventricular; Male; Middle Aged; Radiography, Thoracic; Survivors; Tetralogy of Fallot
PubMed: 32462941
DOI: 10.1177/2324709620926908 -
European Heart Journal. Case Reports Sep 2019Tetralogy of Fallot is a congenital heart defect characterized by pulmonary valve stenosis, ventricular septal defect (VSD), overriding aorta, and right ventricular...
BACKGROUND
Tetralogy of Fallot is a congenital heart defect characterized by pulmonary valve stenosis, ventricular septal defect (VSD), overriding aorta, and right ventricular hypertrophy. In its' extreme form, the pulmonary valve orifice does not develop during organogenesis, resulting in pulmonary atresia. We report a case of catheter ablation of symptomatic atrial fibrillation (AF) in a 37-year-old patient with congenital pulmonary atresia.
CASE SUMMARY
The young man described paroxysmal tachycardia correlating to AF episodes in the previously implanted event recorder. Computed tomography scan described the complex anatomy with congenital pulmonary atresia, VSD, and major aortopulmonary collateral arteries. Electroanatomical mapping revealed typical pulmonary vein electrograms in a hypotrophic left atrium. Modified pulmonary vein isolation was successfully performed and non-excitability of the ablation line was reached. The patient recovered uneventfully and event recorder interrogation showed no AF recurrence after 3 months.
DISCUSSION
Incidence of pulmonary atresia is low. Untreated survival rate is 50% after 1 year and 8% after 10 years. Tachycardia is a major cause of increased morbidity and mortality in patients with cyanotic congenital heart defects and pulmonary vein foci are described as driver for AF. Considerations preceding catheter ablation included pathophysiological mechanism, complex anatomy, atypical left atrium access, and reduced pulmonary perfusion resulting in a hypotrophic left atrium. Pulmonary veins showed typical electrograms, and isolation of pulmonary veins was feasible without adverse events.
PubMed: 31660488
DOI: 10.1093/ehjcr/ytz115