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Giant Coronary Artery Aneurysm Causing Ventricular Tachycardia and Right Ventricular Outflow Tract Obstruction

Alam, Usman; Halpern, Dan G; Donnino, Robert M; Chinitz, Larry A; Small, Adam J
PMID: 38841842
ISSN: 1942-0080
CID: 5665562

Anatomical/Physiological Correlates of Functional Capacity in Adults With Repaired and Nonsevere Coarctation of the Aorta

Ramachandran, Abhinay; Talmor, Nina; Saric, Muhamed; Feinberg, Jodi; Small, Adam J.; Halpern, Dan G.
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.
ISSN: 2772-963x
CID: 5621242

Adult congenital heart disease care in a municipal public health system

Tinsay, Maria Andrea Francesca M; Halpern, Dan G; Feinberg, Jodi L; Vorsanger, Matthew; Keller, Norma; Small, Adam J
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.
PMID: 37909409
ISSN: 1467-1107
CID: 5614462

Anomalous right coronary artery originating from the opposite sinus of Valsalva: Fractional flow reserve and intravascular ultrasound-guided management [Case Report]

Singh, Arushi; Donnino, Robert; Small, Adam; Bangalore, Sripal
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.
PMID: 37724846
ISSN: 1522-726x
CID: 5609442

Anomalous Right Coronary Artery From the Pulmonary Artery Diagnosed in Pregnancy

Bhalla, Natalie R; Small, Adam J
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.).
PMID: 37396323
ISSN: 2666-0849
CID: 5538972

Reproductive Health in Congenital Heart Disease: Preconception, Pregnancy, and Postpartum

Halpern, Dan G; Penfield, Christina A; Feinberg, Jodi L; Small, Adam J
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.
PMID: 37233153
ISSN: 2308-3425
CID: 5543922

Undiagnosed ventricular septal defect with resultant Eisenmenger syndrome presenting with diplopia [Case Report]

Duncan, Ellen; Small, Adam; Sulica, Roxana; Halpern, Dan
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.
PMID: 36456362
ISSN: 1532-8171
CID: 5374132

Congenital anatomy, acquired pathology - A synergistic approach to echocardiographic evaluation of the adult with congenital heart disease [Comment]

Small, Adam J; Bhatla, Puneet; Saric, Muhamed; Halpern, Dan G; Kutty, Shelby
PMID: 36897538
ISSN: 1540-8175
CID: 5448652

Multimodality Imaging of Caval and Coronary Sinus Venous Anomalies [Case Report]

Liebman, Jordan; Bamira, Daniel; Ro, Richard; Vainrib, Alan F; Small, Adam J; Donnino, Robert; Saric, Muhamed
PMID: 36247374
ISSN: 2468-6441
CID: 5356652

Delivery Timing and Associated Outcomes in Pregnancies With Maternal Congenital Heart Disease at Term

Mok, Thalia; Woods, Allison; Small, Adam; Canobbio, Mary M; Tandel, Megha D; Kwan, Lorna; Lluri, Gentian; Reardon, Leigh; Aboulhosn, Jamil; Lin, Jeannette; Afshar, Yalda
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.
PMID: 35943056
ISSN: 2047-9980
CID: 5286822