Adult congenital heart disease care in a municipal public health system.

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.

Anomalous Right Coronary Artery From the Pulmonary Artery Diagnosed in Pregnancy.

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.).

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

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.

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

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.

Isolated Anomalous Right Superior Pulmonary Venous Return to the Inferior Vena Cava.

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.

Combined Transcatheter Tricuspid and Pulmonary Valve Replacement.

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.

Thromboprophylaxis in Adults With Atrio-Pulmonary Fontan.

BACKGROUND: Although aspirin has been compared to warfarin for thromboembolic prophylaxis in the general Fontan population, little is known about the optimal preventative strategy for the atriopulmonary right atrium-pulmonary artery [RA-PA]) Fontan particularly. METHODS: A retrospective cohort study was performed including adult patients identified in the Ahmanson/UCLA Adult Congenital Heart Disease Center database with a history of RA-PA Fontan and use of either aspirin or warfarin as most recent primary prophylaxis against thromboembolism. Primary outcome was incident thromboembolism, defined as space-occupying lesion on imaging consistent with thrombus within the Fontan or pulmonary arterial circuit. Secondary outcomes were death, transplantation, Fontan conversion, and bleeding requiring either transfusion or invasive intervention. Follow-up was terminated upon achievement of a primary outcome or achievement of a secondary outcome other than bleeding. Kaplan-Meier analysis of freedom from thrombosis was performed. RESULTS: Twenty-six patients met inclusion criteria. Thirteen (50%) received aspirin as most recent primary prophylaxis and 13 (50%) received warfarin. Tricuspid atresia was the most common underlying diagnosis (42%), followed by double-inlet left ventricle (38%). Median age at Fontan operation was 8.2 years; median age at prophylaxis initiation was 25.9 years. After six years, the aspirin group had 50% ± 35% freedom from thrombosis and the warfarin group 92% ± 8% ( P = .15). Incidences of secondary outcomes were not significantly different between the groups. CONCLUSION: In this cohort of long-term Fontan survivors with RA-PA Fontan, the risk of thromboembolic complications is high, especially in those taking aspirin rather than warfarin. Larger studies are needed to confirm these findings.

Factors associated with systemic to pulmonary arterial collateral flow in single ventricle patients with superior cavopulmonary connections.

OBJECTIVE: Systemic to pulmonary arterial collateral flow (CollF) is common in single ventricle patients with superior cavopulmonary connections (SCPC), although associations with CollF are not well understood. We previously described a method to quantify CollF by cardiac MRI (CMR). We sought to identify factors associated with CollF in a large cross section of patients with SCPC. METHODS: A retrospective observational cohort study of events from birth to study CMR was performed for all patients with SCPC who had CollF quantified by CMR. RESULTS: CollF was quantified in 96 patients at a median age of 2.6 (IQR 1.9-3.1) years and 2.1 (1.4-2.7) years after SCPC and measured 1.6±0.7 L/min/m(2) (33±11% of aortic flow and 48±16% of pulmonary venous flow). Significantly higher amounts of indices of CollF were associated with: duration of chest tubes (p≤0.05 for all), intensive care unit and hospital length of stay (p≤0.04 for all), higher O2 saturation at Stage 2 discharge (p=0.04 for CollF/aortic), female sex (p≤0.007 for CollF/aortic and CollF/pulmonary venous), and history of a Blalock-Taussig shunt (p<0.04 for CollF and CollF/aortic). Multivariable models were constructed to identify factors independently associated with CollF measures and included: female sex (p≤0.006 for all), O2 saturation at Stage 2 discharge (p=0.013 for CollF/aortic) and total chest tube days (p=0.001 for all). These models explained 20-22% of the variance in the outcomes. CONCLUSIONS: These data support hypotheses that perioperative morbidity and pleural inflammation play a role in CollF development and that CollF affects pulmonary blood flow.

Systemic-to-pulmonary collateral flow, as measured by cardiac magnetic resonance imaging, is associated with acute post-Fontan clinical outcomes.

BACKGROUND: Systemic-pulmonary collateral (SPC) flow occurs commonly in single ventricle patients after superior cavo-pulmonary connection, with unclear clinical significance. We sought to evaluate the association between SPC flow and acute post-Fontan clinical outcomes using a novel method of quantifying SPC flow by cardiac magnetic resonance (CMR) imaging. METHODS AND RESULTS: All patients who had SPC flow quantified by CMR imaging before Fontan were retrospectively reviewed to assess for acute clinical outcomes after Fontan completion. Forty-four subjects were included who had Fontan completion between May 2008 and September 2010. SPC flow prior to Fontan measured 1.5±0.9 L/min/m(2), accounting for 31±11% of total aortic flow and 44±15% of total pulmonary venous flow. There was a significant linear association between natural log-transformed duration of hospitalization and SPC flow as a proportion of total aortic (rho=0.31, P=0.04) and total pulmonary venous flow (rho=0.29, P=0.05). After adjustment for Fontan type and presence of a fenestration, absolute SPC flow was significantly associated with hospital duration ≥7 days (odds ratio [OR]=9.2, P=0.02) and chest tube duration ≥10 days (OR=22.7, P=0.009). Similar associations exist for SPC flow as a percentage of total aortic (OR=1.09, P=0.048 for hospitalization ≥7 days; OR=1.24, P=0.007 for chest tube duration ≥10 days) and total pulmonary venous flow (OR=1.07, P=0.048 for hospitalization ≥7 days; OR=1.18, P=0.006 for chest tube duration ≥10 days). CONCLUSIONS: Increasing SPC flow before Fontan, as measured by CMR imaging, is associated with increased duration of hospitalization and chest tube following Fontan completion.