Project description:Ventricular septal rupture (VSR) is an uncommon but potentially fatal complication of acute myocardial infarction (AMI). The management of VSR is challenging, and its surgical correction is associated with the highest mortality among all cardiac surgery procedures. A 57-year-old man with a history of smoking presented with AMI with a large apical VSR in addition to a large secundum atrial septal defect (ASD). His left ventricular ejection fraction was 30%, and the right ventricle was moderately dilated with normal systolic function. Cardiac catheterization revealed that the left anterior descending artery was diffusely diseased with total mid occlusion, whereas other coronary arteries had non-obstructive disease. This unique combination resulted in distinctive presentation with paradoxically better outcomes. After stabilization, the patient's interventricular septum was reconstructed, and the ASD was closed with a pericardial patch. The post-operative period was uneventful, and the patient was discharged 1 week after surgery. A follow-up echocardiography revealed no residual shunt. Post-myocardial infarction VSR presents differently in patients with pre-existing right ventricular volume overload. In such cases, the absence of significant cardiogenic shock at presentation may result in better surgical outcomes.
Project description:Background Delayed brain development, brain injury, and neurodevelopmental disabilities are commonly observed in infants operated for complex congenital heart defect. Our previous findings of poorer neurodevelopmental outcomes in individuals operated for simple congenital heart defects calls for further etiological clarification. Hence, we examined the microstructural tissue composition in cerebral cortex and subcortical structures in comparison to healthy controls and whether differences were associated with neurodevelopmental outcomes. Methods and Results Adults (n=62) who underwent surgical closure of an atrial septal defect (n=33) or a ventricular septal defect (n=29) in childhood and a group of healthy, matched controls (n=38) were enrolled. Brain diffusional kurtosis imaging and neuropsychological assessment were performed. Cortical and subcortical tissue microstructure were assessed using mean kurtosis tensor and mean diffusivity and compared between groups and tested for associations with neuropsychological outcomes. Alterations in microstructural tissue composition were found in the parietal, temporal, and occipital lobes in the congenital heart defects, with distinct mean kurtosis tensor cluster-specific changes in the right visual cortex (pericalcarine gyrus, P=0.002; occipital part of fusiform and lingual gyri, P=0.019). Altered microstructural tissue composition in the subcortical structures was uncovered in atrial septal defects but not in ventricular septal defects. Associations were found between altered cerebral microstructure and social recognition and executive function. Conclusions Children operated for simple congenital heart defects demonstrated altered microstructural tissue composition in the cerebral cortex and subcortical structures during adulthood when compared with healthy peers. Alterations in cerebral microstructural tissue composition were associated with poorer neuropsychological performance. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT03871881.
Project description:A 50-year-old man presented with an episode of chest pain. Cardiac magnetic resonance revealed the presence of a large ventricular septal aneurysm partially closing a perimembranous ventricular septal defect, prolapsing into the right ventricular outflow tract, and mimicking a mass. We illustrate the diagnostic approach and management of such ventricular septal aneurysms. (Level of Difficulty: Advanced.).
Project description:Background: Patients with congenital heart defects have a well-established risk of neuropsychiatric comorbidities. Inattention and hyperactivity are three to four times more frequent in children with complex congenital heart defects. We have previously shown a higher burden of overall attention deficit/hyperactivity disorder (ADHD) symptoms in adults with simple congenital heart defects as well. However, it is unknown whether the higher burden of ADHD symptoms is mainly driven by hyperactivity, inattention, or both. Methods: The participants [simple congenital heart defect = 80 (26.6 years old), controls = 36 (25.3 years old)] and a close relative for each (n = 107) responded to the long version of the Conners' Adults ADHD Rating Scales questionnaire. Our primary and secondary outcomes are mean T-scores in the ADHD scores and symptom sub-scores. Results: Patients with simple congenital heart defects reported a higher mean T-score at all three DSM-IV ADHD scores (ADHD-combined: 52.8 vs. 44.9, p = 0.007, ADHD-inattention: 55.5 vs. 46.4, p = 0.002, and ADHD-hyperactivity: 49.4 vs. 44.0, p = 0.03) and in all four ADHD symptom sub-scores (inattention/memory problems: 50.3 vs. 44.2, p = 0.001, hyperactivity/restlessness: 49.7 vs. 45.9, p = 0.03, impulsivity/emotional lability: 50.0 vs. 41.3, p = 0.001, and self-esteem problems: 53.8 vs. 46.3, p = 0.003). The results were maintained after the removal of outliers (incongruent responses), albeit the hyperactivity/restlessness ADHD symptom sub-score lost significance. Self- and informant ratings differed significantly on the ADHD-inattention score for the congenital heart defect group, where informants rated the ADHD-inattention scores better than the congenital heart defect patients rated themselves. Conclusions: Patients with a simple congenital heart defect have a higher symptom burden across all ADHD scores and all symptom sub-scores. The higher burden of ADHD is driven by both inattention and hyperactivity symptoms, though the inattention symptoms seem more prominent. Close relatives were less aware of the inattention symptoms than the congenital heart defect patients themselves. Routine screening for ADHD symptoms may be warranted to facilitate adequate help and guidance as these symptoms are easily overlooked. Clinical Trial Registration: www.ClinicalTrials.gov, identifier: NCT03871881.
Project description:In our practice, we have been struck by the presence of central hypoxemia in individuals with congenitally corrected transposition of the great arteries (CCTGA) with an atrial septal defect (ASD) as the only association, despite existence of normal pulmonary artery pressures. This right to left shunting at the atrial level occurs only in those patients where there is additional presence of apicocaval juxtaposition (ACJ). Patients with CCTGA but without ACJ manifest only left to right shunting at the level of the ASD. We present a case series of patients with CCTGA with intact ventricular septum and incomplete atrial septum with a possible basis for this phenomenon.
Project description:Congenital diveticula and aneurysm of the heart are rare and most often located at the apex of the left ventricle. They pose a significant risk for cardiac failure and arrhythmias. In contrast, nonapical diverticula of the right ventricle (RV) have a much more benign course. We present a child with Trisomy 21, atrioventricular septal defect, and large nonapical diverticulum of the RV that was neither addressed during surgery nor needed any medical treatment during 1-year follow-up.
Project description:A 22-year-old male presented with recurrent stroke, central cyanosis, and dyspnea. Transesophageal echocardiography and cardiac catheterization revealed bidirectional shunt flow through atrial septal defect (ASD) without pulmonary arterial hypertension. The orifice of inferior vena cava facing towards ASD opening led partially right to left shunt resulting in cyanosis with normal pulmonary arterial pressure.
Project description:A 63-year-old man presented with generalized fatigue, chills, malaise, dyspnea, intermittent fevers, and 50-pound weight loss of 4 months' duration. Blood cultures were positive for pan-sensitive Streptococcus anginosus. Transesophageal echocardiography showed an 11 mm × 3 mm mobile mass attached to the mitral valve, a 16 mm × 16 mm mobile mass attached to the pulmonary valve, and a small membranous ventricular septal defect. The patient received 12 weeks of intravenous (IV) antibiotics with eventual resolution of the masses. Multi-valve endocarditis involving both the left and right chambers is rarely reported without prior history of IV drug use or infective endocarditis. Our case emphasizes the importance of careful assessment for ventricular septal defects or extra-cardiac shunts in individuals who present with simultaneous right and left-sided endocarditis.
Project description:Takotsubo Syndrome is a transient condition characterized by left ventricular systolic dysfunction with apical akinesis/dyskinesis and ballooning. Although the prognosis with medical management is excellent in most cases, rare cases of serious complications can occur. We present here a case of a 71-year-old woman presenting with acute decompensated heart failure with initial findings consistent with a myocardial infarction, who was found instead to have an acute ventricular septal defect as a complication of Takotsubo Syndrome.