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This paper presents the case of a 30-year-old man who was diagnosed with an apical-lateral wall left ventricular aneurysm with scarring, prominent left ventricular trabeculations, and mildly diminished systolic function. Working diagnosis was a congenital left ventricular aneurysm in the setting of left ventricular noncompaction, yet with a questionable defect of the pericardium.

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Arrhythmias are highly prevalent in adults with congenital heart disease. For the clinician caring for this population, an understanding of pathophysiology, diagnosis, and management of arrhythmia is essential. Herein we review the latest updates in diagnostics and treatment of tachyarrhythmias and bradyarrhythmias, all in the context of congenital anatomy, hemodynamics, and standard invasive palliations for congenital heart disease.

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Specialty care is associated with improved outcomes for adults with adult CHD and must be extended to the underserved. A retrospective cohort study was performed to describe the provision of care to adult CHD patients in America's largest municipal public health system including patient demographics, diagnostic and therapeutic procedures, and adherence to guideline-recommended surveillance. We identified 229 adult CHD patients aged >18 years through electronic medical records. The most common diagnoses were atrial septal defect, ventricular septal defect, patent ductus arteriosus, and valvular pulmonary stenosis. In total, 65% had moderate or greater anatomic complexity. A large number of patients were uninsured (45%), non-white (96%), and non-English speaking (44%). One hundred forty-six patients (64%) presented with unrepaired primary defects. Fifty eight patients underwent primary repair during the study period; 48 of those repairs were surgical and 10 were transcatheter. Collaboration with an affiliated Comprehensive Care Center was utilised for 28% of patients. A high proportion of patients received adult CHD speciality visits (78%), echocardiograms (66%), and electrocardiograms (56%) at the guideline-recommended frequency throughout the study period. There was no significant difference in the rate of adherence to guideline-recommended surveillance based on insurance status, race/ethnicity, or primary language status. The proportion of patients who had guideline-recommended adult CHD visits, echocardiograms, and electrocardiograms was significantly lower for those with more advanced physiological stages. These results can inform the provision of adult CHD care in other public health system settings.

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There remains significant controversy in the risk stratification and management of patients with anomalous right coronary artery originating from the opposite sinus (R-ACAOS). We present the case of a patient with an inferior ST-elevation myocardial infarction, found to have R-ACAOS and severe atherosclerotic right coronary artery disease, treated with fractional flow reserve and intravascular ultrasound-guided percutaneous coronary intervention.

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A 39-year-old gravida 2, para 1 woman presented for evaluation of palpitations in pregnancy. She was found to have supraventricular tachycardia. The initial echocardiogram demonstrated a color Doppler signal in the proximal pulmonary artery with diastolic-dominant flow, suggestive of coronary flow. She received a diagnosis of anomalous right coronary artery from the pulmonary artery. Her arrhythmia was managed medically. We discuss techniques for risk stratification of cardiac lesions in pregnancy and for the management of anomalous coronary origin from the pulmonary artery. (Level of Difficulty: Intermediate.).

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The prevalence of congenital heart disease (CHD) in pregnancy is rising due to the improved survival of patients with CHD into childbearing age. The profound physiological changes that occur during pregnancy may worsen or unmask CHD, affecting both mother and fetus. Successful management of CHD during pregnancy requires knowledge of both the physiological changes of pregnancy and the potential complications of congenital heart lesions. Care of the CHD patient should be based on a multidisciplinary team approach beginning with preconception counseling and continuing into conception, pregnancy, and postpartum periods. This review summarizes the published data, available guidelines and recommendations for the care of CHD during pregnancy.

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Ventricular septal defect (VSD) is the most common congenital heart lesion among children. In most cases, however, it is identified and corrected in childhood, before long-term sequelae such as pulmonary hypertension develop. In this case report, we present a young man with an undiagnosed VSD with consequent Eisenmenger syndrome who initially presented to medical attention with diplopia found to be caused by cerebral infarcts.

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Background: There is limited data regarding the interplay of anatomic and physiologic parameters with exercise capacity in adults with native or recurrent nonsevere coarctation of the aorta (CoA). Objectives: The objective of this study was to use exercise stress echocardiography and cardiac magnetic resonance imaging to identify anatomic and physiologic correlates of exercise capacity in these patients. Methods: We conducted a single-center retrospective analysis of 54 adults with nonsevere CoA (repaired or unrepaired) followed at our institution between 2015 and 2020. Resting coarctation gradients were obtained using echocardiography. Exercise gradients and functional capacity were assessed using exercise stress echocardiography. Aorta anatomy was obtained using magnetic resonance imaging. Results: Coarctation-to-diaphragm ratio correlated with minutes of exercise (r = 0.56, P < 0.01) and metabolic equivalents (r = 0.49, P < 0.01). These relationships remained significant after controlling for use of beta-blockers, valvular disease, and type of coarctation repair. Minutes of exercise correlated with mean resting gradients (r = -0.39, P < 0.05). Coarctation-to-diaphragm ratio correlated with peak and mean resting gradients (r = -0.34, P < 0.05; r = -0.48, P < 0.01). Patients with coarctation-to-diaphragm ratio ≤0.7 achieved fewer metabolic equivalents (11.1 ± 1.9 vs 12.8 ± 2.2, P < 0.05) and minutes of exercise (10.3 ± 2.0 vs 12.6 ± 2.7, P < 0.05). Conclusions: In patients with nonsevere native or recurrent CoA, reduced exercise capacity is correlated with coarctation severity by anatomic size and gradients. Those with a coarctation-to-diaphragm ratio ≤0.7 may represent a subset of patients with nonsevere CoA whose clinical symptoms are only elicited with exercise stress testing. Exercise stress testing and cross-sectional imaging may help identify those who could be considered for earlier coarctation intervention.

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Background Current recommendations for delivery timing of pregnant persons with congenital heart disease (CHD) are based on expert opinion. Justification for early-term birth is based on the theoretical concern of increased cardiovascular stress. The objective was to evaluate whether early-term birth with maternal CHD is associated with lower adverse maternal or neonatal outcomes. Methods and Results This is a retrospective cohort study of pregnant persons with CHD who delivered a singleton after 37 0/7 weeks gestation at a quaternary care center with a multidisciplinary cardio-obstetrics care team between 2013 and 2021. Patients were categorized as early-term (37 0/7 to 38 6/7 weeks) or full-term (≥39 0/7) births and compared. Multivariable logistic regression was conducted to calculate the adjusted odds ratio for the primary outcomes. The primary outcomes were composite adverse cardiovascular, maternal obstetric, and adverse neonatal outcome. Of 110 pregnancies delivering at term, 55 delivered early-term and 55 delivered full-term. Development of adverse cardiovascular and maternal obstetric outcome was not significantly different by delivery timing. The rate of composite adverse neonatal outcomes was significantly higher in early-term births (36% versus 5%, P<0.01). After adjusting for confounding variables, early-term birth remained associated with a significantly increased risk of adverse neonatal outcomes (adjusted odds ratio 11.55 [95% CI, 2.59-51.58]). Conclusions Early-term birth for pregnancies with maternal CHD was associated with an increased risk of adverse neonatal outcomes, without an accompanying decreased rate in adverse cardiovascular or obstetric outcomes. In the absence of maternal or fetal indications for early birth, induction of labor before 39 weeks for pregnancies with maternal CHD should be reserved for routine obstetrical indications.

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Partial anomalous pulmonary venous connection is a rare congenital abnormality. We present images from an atypical case of isolated anomalous right superior pulmonary venous return to the inferior vena cava without other cardiac abnormalities.

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We report a case of Streptococcus mutans multivalvular infective endocarditis complicated by aortic root abscess and septic emboli in a 19-year-old male with a bicuspid aortic valve. This case illustrates the progression of untreated subacute bacterial endocarditis and highlights the importance of ongoing clinical suspicion for infective endocarditis in patients with underlying valvular defects.

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This report describes a young adult man presenting with subarachnoid hemorrhage secondary to an intracranial aneurysm who was found to have a short-segment type B interrupted aortic arch. We describe the clinical presentation, evaluation, and management of this patient and highlight imaging findings and percutaneous repair of the aneurysm and interrupted aortic arch. (Level of Difficulty: Intermediate.).

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BACKGROUND: Acquired pulmonary vein stenosis (PVS) is an infrequent complication of atrial fibrillation ablation that is often misdiagnosed due to predominant respiratory symptoms. It can result in pulmonary venous hypertension, with varying presentations, ranging from shortness of breath to haemoptysis. CASE SUMMARY: We report two patients with a history of paroxysmal atrial fibrillation treated with radiofrequency ablation and pulmonary vein (PV) isolation, who subsequently developed PVS. Case 1 initially presented with indolent symptoms of shortness of breath and cough. He was initially diagnosed with and treated for pneumonia. In contrast, Case 2 presented with massive haemoptysis, requiring intubation and intensive care unit admission. Both patients were eventually diagnosed with PVS by computed tomography. They were treated with PV angioplasty and stenting. DISCUSSION: While previously limited to the congenital heart disease population, PVS is occurring more frequently now in adult patients as a complication of ablation procedures. It is most effectively treated with angioplasty and stent implantation but has a high rate of recurrence.

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BACKGROUND: Patients with the Fontan circulation lack a subpulmonary ventricular pump and thus the main driver for pulmonary blood flow is a high central venous pressure. Peripheral venous pressure (PVP) measurement has been shown to be a reproducible and fairly accurate surrogate for central venous pressure (CVP), but not specifically for the adult Fontan circulation. This study aims to determine the relationship of PVP to CVP in adult Fontan patients. METHODS: All adult patients (≥18 yo) with a Fontan circulation undergoing cardiac catheterization were included. Both CVP and PVP were measured during the catheterization. The relationship between the peripheral venous and central venous pressures was assessed using simple linear regression and the Bland-Altman plot analysis for differences. RESULTS: Thirty-eight adult Fontan patients (mean age 30.7 ± 8.5, range 18-52 years) undergoing 43 cardiac catheterizations were analyzed. The mean CVP was 17.3 +/- 4.7 mmHg. The mean PVP was 18.4 +/- 5 mmHg. CVP and PVP were highly correlated, with an R2 value of 0.83 (p < 0.001). The CVP can be estimated with PVP measurements using the formula CVP = (0.86 * PVP) + 1.3. A Bland-Altman plot for PVP and CVP demonstrated that the PVP overestimated CVP by a mean of 1.2 mmHg, with a 95% limit of agreement of -5.2 mmHg to 2.8 mmHg. CONCLUSIONS: In adult Fontan patients, measuring PVP is a reliable, less-invasive, and accurate method of estimating the CVP during cardiac catheterization procedures. These findings may enable outpatient monitoring of Fontan hemodynamics.

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BACKGROUND: For patients with combined tricuspid and pulmonary valve disease, operative intervention carries high mortality risk. Published reports of combined transcatheter tricuspid and pulmonary valve replacement have been limited to single cases. METHODS: A retrospective chart review was performed including all patients undergoing combined transcatheter tricuspid and pulmonary valve replacement at the Ahmanson/UCLA Adult Congenital Heart Disease Center. RESULTS: Combined transcatheter tricuspid and pulmonary valve replacement was undertaken in five adult patients (median age: 46 years; range: 24-64 years). Three had congenital heart disease and two had carcinoid syndrome. Four patients had previous surgical tricuspid valve replacement and one had a surgical incomplete annuloplasty ring. Four patients had previous surgical pulmonary valve replacement and one had a right ventricle-to-pulmonary artery homograft conduit. Two patients underwent Medtronic Melody valve implantation in both tricuspid and pulmonary positions and three underwent Edwards Sapien S3 implantation in both tricuspid and pulmonary positions. Valve implantation was successful in all. Follow-up ranged from 0.9 to 3.0 years. One patient underwent redo transcatheter tricuspid valve replacement 12 months after the first transcatheter intervention for progressive regurgitation of a Melody valve. This patient died 2.5 years after combined valve placement of complications from refractory heart failure. The remaining patients were alive and free of valve reintervention at follow-up. CONCLUSIONS: Combined transcatheter tricuspid and pulmonary valve replacement can be performed successfully, with an acceptable complication rate. This strategy is a feasible option for appropriately selected patients.

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