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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 -
Fetal and Pediatric Pathology Feb 2019The main characteristics of absent pulmonary valve syndrome (APVS) include the absence or hypoplasia of the pulmonary valve, stenosis of the pulmonary valve annulus, and... (Review)
Review
INTRODUCTION
The main characteristics of absent pulmonary valve syndrome (APVS) include the absence or hypoplasia of the pulmonary valve, stenosis of the pulmonary valve annulus, and aneurysmal dilatation of the pulmonary trunk and its branches. In the more common type 1, the tetralogy of Fallot-like type, there is a ventricular septal defect, overriding aorta, pulmonary arterial dilatation, and absence of ductus arteriosus, The second type has an intact ventricular septum, less pulmonary artery dilatation, and a patent ductus arteriosus, with or without tricuspid atresia.
CASE REPORT
This APVS had an intact ventricular septum with an absent ductus arteriosus.
CONCLUSION
The APVS with intact ventricular septum with an absent ductus arteriosus may represent a third type of APVS.
Topics: Fetus; Heart Defects, Congenital; Humans; Male; Pulmonary Valve
PubMed: 30661433
DOI: 10.1080/15513815.2018.1529066 -
European Journal of Cardio-thoracic... Jul 2019Tetralogy of Fallot is characterized by anterocephalad deviation of the outlet septum, along with abnormal septoparietal trabeculations, which lead to subpulmonary...
OBJECTIVES
Tetralogy of Fallot is characterized by anterocephalad deviation of the outlet septum, along with abnormal septoparietal trabeculations, which lead to subpulmonary infundibular stenosis. Archives of retained hearts are an important resource for improving our understanding of congenital heart defects and their morphological variability. This study aims to define variations in aortic override, coronary arterial patterns and ventricular septal defects in tetralogy of Fallot as observed in a morphological archive, highlighting implications for surgical management.
METHODS
The Birmingham Children's Hospital archive contains 211 hearts with tetralogy of Fallot, of which 164 were analysed [69 (42.1%) unrepaired and 95 (57.9%) operated specimens]. A detailed morphological and geometric analysis was performed using a rigorous 5-layer review process.
RESULTS
Anomalies were observed in the orifices, origins and course of the coronary arteries: 20 hearts (13.0%) had more than 2 orifices and 3 hearts (1.9%) had a single orifice. In 7 hearts (4.3%), a coronary artery crossed the right ventricular outflow tract. The extent of aortic override ranged from 31.0% to 100% (median of 59.5%). The ventricular septal defect was most often perimembranous (139, 84.8%), but we also found muscular (14, 8.5%), atrioventricular (7, 4.3%) and doubly committed juxta-arterial (2, 1.2%) variants.
CONCLUSIONS
Anatomical variations are common and can impact surgical management. Anomalous coronary arteries may require a conduit rather than a transannular patch. Variability in aortic override determines the size of patch used to baffle blood to the aorta. The type of ventricular septal defect affects patch closure and the risk of postoperative conduction defects.
Topics: Adolescent; Child; Child, Preschool; Cohort Studies; Coronary Vessel Anomalies; Coronary Vessels; Female; Humans; Infant; Infant, Newborn; Male; Tetralogy of Fallot
PubMed: 30657877
DOI: 10.1093/ejcts/ezy474 -
Paediatric Anaesthesia May 2019Tetralogy of Fallot (TOF) has four anatomic features: right ventricular hypertrophy (RVH), ventriculoseptal defect (VSD), overriding aorta and right ventricular outflow... (Review)
Review
Tetralogy of Fallot (TOF) has four anatomic features: right ventricular hypertrophy (RVH), ventriculoseptal defect (VSD), overriding aorta and right ventricular outflow tract obstruction (RVOT) with an occurrence of 3.9 /10,000 births. The pathophysiologic effects in TOF are largely determined by the degree of RVOT and not the VSD. Intra-operative anesthetic management is also dependent on the degree of RVOT obstruction and influenced by the extent of surgical RVOT repair.
Topics: Cardiac Surgical Procedures; Child; Child, Preschool; Humans; Infant; Infant, Newborn; Tetralogy of Fallot
PubMed: 30592107
DOI: 10.1111/pan.13569 -
Echocardiography (Mount Kisco, N.Y.) Feb 2019Aortopulmonary window is a rare conotruncal defect that is often associated with other congenital heart defects. We present a patient with a previously unreported...
BACKGROUND
Aortopulmonary window is a rare conotruncal defect that is often associated with other congenital heart defects. We present a patient with a previously unreported combination of aortopulmonary window with tetralogy of Fallot with an absent conal septum.
CASE PRESENTATION
A term, 2.4 kg newborn male infant presented at a community hospital with cyanosis unresponsive to supplemental oxygen. Transthoracic echocardiography demonstrated a conotruncal defect with a large conoventricular ventricular septal defect and an over-riding, dysplastic aortic valve. The main pulmonary artery (MPA) appeared to arise from left facing sinus of the aortic valve, with confluent yet hypoplastic right and left branch pulmonary arteries. There was no evidence of prograde flow into the MPA in systole, though there did appear to be retrograde flow in diastole from the patent ductus. The patient underwent multiple advanced imaging studies, and the diagnosis was not fully elucidated. Postmortem examination demonstrated morphology consistent with Tetralogy of Fallot with the absence of the conal septum. There were two distinct semilunar valves in fibrous continuity with an aortopulmonary window immediately cephalad to the valve.
DISCUSSION
The rare combination of defects and the patient's size made the anatomic diagnosis by conventional imaging challenging. However, retrospective review of imaging studies did demonstrate anatomic features seen by direct examination of the specimen.
Topics: Animals; Aorta; Echocardiography; Fatal Outcome; Heart Septal Defects, Ventricular; Humans; Infant, Newborn; Male; Pulmonary Artery; Tetralogy of Fallot
PubMed: 30585348
DOI: 10.1111/echo.14243 -
American Journal of Physiology. Heart... Dec 2018Studies have suggested the effect of blood flow forces in pathogenesis and progression of some congenital heart malformations. It is therefore of interest to study the...
Studies have suggested the effect of blood flow forces in pathogenesis and progression of some congenital heart malformations. It is therefore of interest to study the fluid mechanic environment of the malformed prenatal heart, such as the tetralogy of Fallot (TOF), especially when little is known about fetal TOF. In this study, we performed patient-specific ultrasound-based flow simulations of three TOF and seven normal human fetal hearts. TOF right ventricles (RVs) had smaller end-diastolic volumes (EDVs) but similar stroke volumes (SVs), whereas TOF left ventricles (LVs) had similar EDVs but slightly increased SVs compared with normal ventricles. Simulations showed that TOF ventricles had elevated systolic intraventricular pressure gradient (IVPG) and required additional energy for ejection but IVPG elevations were considered to be mild relative to arterial pressure. TOF RVs and LVs had similar pressures because of equalization via ventricular septal defect (VSD). Furthermore, relative to normal, TOF RVs had increased diastolic wall shear stresses (WSS) but TOF LVs were not. This was caused by high tricuspid inflow that exceeded RV SV, leading to right-to-left shunting and chaotic flow with enhanced vorticity interaction with the wall to elevate WSS. Two of the three TOF RVs but none of the LVs had increased thickness. As pressure elevations were mild, we hypothesized that pressure and WSS elevation could play a role in the RV thickening, among other causative factors. Finally, the endocardium surrounding the VSD consistently experienced high WSS because of RV-to-LV flow shunt and high flow rate through the over-riding aorta. NEW & NOTEWORTHY Blood flow forces are thought to cause congenital heart malformations and influence disease progression. We performed novel investigations of intracardiac fluid mechanics of tetralogy of Fallot (TOF) human fetal hearts and found essential differences from normal hearts. The TOF right ventricle (RV) and left ventricle had similar and elevated pressure but only the TOF RV had elevated wall shear stress because of elevated tricuspid inflow, and this may contribute to the observed RV thickening. TOF hearts also expended more energy for ejection.
Topics: Adult; Female; Fetal Heart; Hemodynamics; Humans; Infant, Newborn; Models, Cardiovascular; Myocardial Contraction; Pregnancy; Tetralogy of Fallot
PubMed: 30216114
DOI: 10.1152/ajpheart.00235.2018