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Introduction: After the initial surgical repair of tetralogy of Fallot (rTOF), right ventricular outflow tract dysfunction is common, with pulmonary stenosis (PS), regurgitation, or both. Obese adults with rTOF have worse biventricular systolic function, and greater post-operative pulmonary valve replacement morbidity than non-obese patients. Transcatheter PVR (TPVR) is used increasingly, though no studies have examined the impact of body mass index (BMI) on morbidity and hemodynamics in adults with rTOF and subsequent TPVR. Hypothesis: BMI affects outcomes of transcatheter PVR in adults with TOF. Methods: This was a 10 yr, single center, retrospective review of adults (>18 yo) with rTOF who underwent TPVR. The cohort was split into 2 groups based on BMI at time of TPVR. Group A (Grp A): normal and overweight (BMI Results: There were 81 adults, 42 (52%) normal, 18 (22%) overweight (Grp A) and 21 (26%) obese (Grp B). Mean follow up was 6.4 + 3.1 yrs. With most recent post-TPVR echocardiogram, there was no significant difference in LV or RV size and function across groups. Compared to Grp A, Grp B patients were more likely to develop any degree of PS (mild, moderate, or severe) following TPVR (69% vs 94%, respectively, p = 0.032). An ROC curve analysis demonstrated BMI ≥ 26.3 had a low sensitivity (45%), but good specificity (89%) for predicting PS post TPVR. There was no difference between groups requiring re- intervention for PVR. Conclusion: This is the first study to show greater BMI affects valve function in adults with rTOF following TPVR. BMI >26 was associated with a greater risk of PS in follow up echocardiograms after TPVR. Obesity is an epidemic in the US and patient prosthesis mismatch may be an issue when considering TPVR. Further studies are indicated to determine the long-term effects of BMI on TPVR and the need for re-intervention in adults with TOF.

Introduction: Athletic cardiac adaptation is associated with structural changes that can overlap with disease states, unnecessarily limiting sports participation. Cardiovascular magnetic resonance (CMR) is useful in athlete’s heart and provides myocardial tissue characterization by T1 and T2 mapping. Hypothesis: CMR in competitive athletes will show abnormal T1 and T2 mapping due to intense exercise induced myocardial edema that can overlap with myocarditis. Methods: CMR data including left ventricular ejection fraction (LVEF) and T1/T2 maps were collected using standardized protocols on a 1.5 T scanner and compared between competitive athletes (N = 18, 83% male, median age 20 years), clinical myocarditis (N = 42, 71% male, median age 23 years) and controls (N = 35, 86% male, median age 22 years) between 2016-2020. T2 values of Results: Figure 1 (panel A) shows participating sport and indications for CMR in athletes. There were 11 athletes (61%) with elevated T2 values (>59 ms), of which 9 (82%) were without clinical myocarditis. Average T2, native T1, ECV, and LVEF are shown in panels B-E. T2 values were highest in myocarditis, followed by athletes and controls (p = 0.001). ECV was higher in myocarditis compared to athletes (p = 0.002). LGE was present in 8/18 athletes and 41/42 myocarditis patients. 6 athletes had follow-up CMR after a period of deconditioning, with 3 (50%) demonstrating an improvement in T2 values and LGE. Conclusions: To conclude, we demonstrate abnormalities on T2 mapping in athletes consistent with myocardial edema or inflammation. Changes in T2 may be related to intense training. Additional studies are required to prospectively evaluate athletes for normative T1 and T2 mapping values, relationship to training, and their correlation with LGE.

Introduction: Pregnancy is contraindicated in women with pulmonary hypertension (PH), yet many still decide to pursue pregnancy. Hypothesis: We hypothesized improved maternal mortality with PH at our center’s cardio-obstetrics program and sought to identify factors to estimate the risk of major adverse cardiac events (MACE). Methods: Pregnant women with right ventricular systolic pressure (RVSP) ≥35 mmHg or tricuspid regurgitant velocity > 2.8 m/s on transthoracic echocardiogram (TTE) were identified. Women with intermediate to high probability PH by ESC criteria (TTE or catheterization, n = 70) were classified using the 6 th World Society of PH definitions. Results: In 70 women with PH (30 ± 6 years-old, RVSP 52 ± 16 mmHg) there were 12 (17%) with WHO Group 1 PH, 45 (64%) with Group 2 PH, 4 (6%) with Group 3 PH and 9 (13%) with Group 5 PH (Figure A). Baseline characteristics were similar except: Group 1 PH had 83% on prostacyclin (PC) therapy, higher RVSP (78 ± 20 mmHg vs. Groups 2 (46 ± 9), 3 (44 ± 2 mmHg) and 5 PH (48 ± 10mmHg), p50 mmHg (95% CI 1.3-10, p = 0.02) and LVEF Conclusion: To conclude, in a large single center cohort we report 100% 1-year survival in Groups 1, 3, and 5 PH, with most Group 1 PH patients on PC therapy and under cardio-obstetrics care. We identify Group 2 PH as an under-recognized group for adverse outcomes in pregnancy, with NYHA FC, pre-eclampsia, RVSP >50 mmHg and LVEF

Introduction: A dilated main pulmonary artery (MPA) seen in congenital heart disease (CHD) or pulmonary arterial hypertension (PAH) is a rare cause of angina due to left main coronary artery (LMCA) compression. Case: A 49 year old male with no prior cardiac history presented with substernal chest pain. He reported exercise intolerance for several years. On exam he had an oxygen saturation of 85%, loud P2, fixed split second heart sound and ejection click. Electrocardiogram showed right ventricular (RV) hypertrophy and no significant ST changes. Laboratory data revealed a negative troponin. An enlarged main and right pulmonary artery (RPA) (Figure 1A) was seen on computed tomography (CT) chest angiography for pulmonary embolism (CTPE) and on echocardiography he had a severely dilated RV with decreased function (Figure 1B,1C). Cardiac catheterization showed a step up in saturations from 56 to 71%, Qp:Qs of 1.5 mean MPA pressure of 53 mmHg, pulmonary capillary wedge pressure of 11mmHg and pulmonary vascular resistance (PVR) of 7WU. A slit-like narrowing of LMCA ostium was seen on coronary angiography and intravascular ultrasound (Figure 1D). He was diagnosed with a sinus venosus defect, anomalous right upper pulmonary venous drainage and suspicion for extrinsic compression of LMCA by the dilated RPA that was confirmed on cardiac CT (Figure 1E-I). He was started on triple therapy for PAH with a significant reduction in PA pressures (mean 36 mmHg and PVR 2.1 WU). Percutaneous stenting of the LMCA was deferred and he underwent successful sinus venosus defect repair with a 5mm fenestrated patch, baffling of the anomalous pulmonary veins and a 32 mm interposition graft in the MPA with reduction plasty of MPA/RPA. Conclusion: This is an unusual presentation of unrepaired CHD with angina due to a compressed LMCA from a dilated pulmonary artery. Treatment of the underlying PAH as well as repair of the defect including reduction plasty of the MPA is a consideration for symptomatic relief.

Introduction: COVID19 pandemic has resulted in drastic changes in healthcare delivery with increase in use of telemedicine. There is limited data on patient experience with telemedicine in adults with congenital heart disease (ACHD). Hypothesis: We hypothesized that ACHD patients would prefer in-person clinic visits over telemedicine, and this would be especially true for patients > 65 years who may be at a higher risk of experiencing technical limitations. Methods: We conducted a nurse administered telephone survey (Figure 1A) based on Agency for Healthcare Research and Quality recommendations to assess patient experience after ACHD telemedicine visits from March 2020 to June 2020. Results: Of 216 patients who had ACHD telemedicine visits, 136 (63%) agreed to participate in the survey. Mean age was 45 + 18 years, majority (65%) being video encounters. Most (98%) said that the telemedicine visit was successful in addressing their healthcare needs. Only 21 (15%) patients reported technical issues. Most patients (76%) were likely to choose telemedicine in the future instead of an in-person clinic visit provided testing was done separately. Given a choice between in-person clinic visit and telemedicine: 103 (76%) preferred telemedicine, 25 (18%) preferred in-person clinic visits, and 8 (6%) had no preference (Figure1B). Of the 25 patients over 65 years, 19 (76%) would choose telemedicine over the in-person clinic, and only one patient reported technical difficulties. Conclusion: In conclusion, patients reported a positive experience with ACHD telemedicine, which was true even among elderly patients. Technical limitations were infrequent even amongst the elderly. Majority of patients felt that their healthcare needs were addressed and would consider telemedicine over clinic visits if given a choice. This data is vital as payors move away from supporting telemedicine and hospitals restructure, with and without ongoing COVID 19 concerns.

Introduction: Pulmonary hypertension (PH) diagnosed by elevated right ventricular systolic pressure (RVSP) is present in 6-11% of patients with sickle cell disease (SCD) and is associated with increased mortality. PH, independent of SCD, is associated with increased maternal mortality. While there are reports of adverse outcomes during pregnancy in patients with SCD, pregnancy outcomes of patients with SCD and PH have not been well studied. Methods: Using our institutional electronic data warehouse we searched for pregnant women who had an echocardiogram between November 1, 2011 and April 30, 2018, and met criteria for pulmonary hypertension with report of either RVSP > 35 mmHg or tricuspid regurgitant (TR) velocity of more than 2.8 m/s. We assessed maternal and fetal outcomes in this group of patients. Results: We identified 136 women with elevated RVSP, of which 7 (5%) had SCD (mean maternal age 29 ± 3 years, mean RVSP 47 ± 11 mmHg, mean TR velocity 3.1 ± 0.4 m/s). RVSP was known to be elevated before pregnancy in 5 (71%). There were no maternal deaths during pregnancy or up to 1-year post-partum. Thromboembolic complications occurred in 3 (43%); including 1 deep vein thrombosis with presumed pulmonary embolism (PE) at 20 weeks gestational age (GA), 1 catheter associated right atrial thrombus at 22 weeks GA, and 1 PE at 16 weeks GA. There were no heart failure complications. Cesarean section occurred in 5 (71%), all for obstetric indications. There were no fetal or neonatal deaths. Pre-eclampsia occurred in 2 (28%). The average gestational age was 35 ± 4 weeks. Preterm birth and low birth weight occurred in 3 (43%) patients. Conclusion: In conclusion, there was no maternal mortality or heart failure in pregnant women with SCD and elevated RVSP at our high-volume center, however 3 of 7 patients (43%) had thromboembolic complications. Further studies of women with PH and SCD are needed to assess the thromboembolic risk during pregnancy.

For more than half a century, growing numbers of practitioners and clinician-scientists have created systems for and practiced healthcare delivery within centers of expertise for young and older adults with congenital heart disease (CHD). To date, this has been driven largely by energy, enthusiasm, and shared mission without supportive data demonstrating the benefit of such adult CHD (ACHD) care programs in the lives of patients and families served by this developing field. Now, for the first time, Mylotte and colleagues1 present in this issue of Circulation good evidence that there is a survival advantage to ACHD patients being cared for by ACHD specialists. This confirms what those working in this field had long assumed and hoped but to date could not substantiate. Article see p 1804 The authors used their own extensively validated, province-wide, administrative longitudinal database that has been highly effective in demonstrating the demography and epidemiology of ACHD care in Quebec, Canada, as a model for North America. Their study included data from 1990 to 2005. They performed an elegant combination of time-series analysis together with case-control studies that included year-dependent survival and center-dependent survival so as to determine mortality trends within a highly monitored and geographically stable group of adults with CHD. A number of important findings emerged. Specialized ACHD care was independently associated with reduced mortality. Among patients who died, just under half died of cardiovascular diseases. Other causes of death included neoplasms (≈18%), respiratory disease (≈9%), and diseases of other systems …

Adult congenital heart disease (ACHD) patients (pts) are an underserved, complex, rapidly expanding population and, despite surpassing the volume of pediatric CHD pts, neither IM or pediatric cardiology fellowship fully prepares physicians to care for this group. The diagnostic studies recommended, technical skills required, and unique data interpretation requires specialized training in both disciplines. Therefore, we sought to determine whether pts referred to our ACHD program underwent appropriate and complete testing prior to referral and evaluated the financial impact of unnecessary and repeat testing. Methods: We reviewed diagnostic testing before and after referral to the ACHD program for consecutive new pts (>18 yo). All cardiovascular testing prior to referral and subsequent testing were recorded. Unnecessary testing was defined as those performed outside of guidelines or inappropriate for their CHD. Incomplete testing was defined as that which required repeating. Financial cost of unnecessary and repeat testing was estimated based on usual insurance billing practice. Results: From 2/06 to 4/07, 200 new pts were referred to the ACHD program, mean age 33.2 ± 12.7 yrs (range 18.1–72.9 yrs). Common diagnoses included ASD (17%), TOF (14%), bicuspid aortic valve (11%) and COA (10%) with 81% classified as moderate or complex CHD. Unnecessary testing consisted of 35 stress studies (25 nuclear and 10 echo), 5 TEEs, and 14 cardiac caths. Repeat studies for incomplete testing were necessary in 65/81 TTEs (80%), 9/39 TEEs (23%), 0/5 cardiac CTs, 8/12 CMRs (67%) and 16/31 caths (51%). Overall,105/200 pts (52%) endured unnecessary or repeat testing carrying a conservative financial burden of $456,980; 916 patient hours: and 114 patient work days lost. Conclusions: Diagnostic testing in ACHD requires specialized training from technician to physician. Repeat testing was necessary in 58% of studies, and 52% of the pts were affected by unnecessary or repeat testing. Therefore, ACHD pts frequently endure unnecessary and repeat testing which carries a financial burden to the patient and health care system and medical risk to the patient. Adults with moderate and complex CHD should be referred to appropriately trained ACHD providers prior to diagnostic testing.

A 37-year-old, physically active man presented for evaluation of nonexertional chest pain. He described it as sharp, left-sided, and nonradiating. The review of systems was unremarkable. He had had no previous thoracic surgery. Physical examination revealed a nonpalpable apical impulse, with no other abnormal findings. A chest radiograph (Figure 1) demonstrated a prominent pulmonary artery shadow, with leftward and posterior rotation of the cardiac …

A17-year-old gravida II, para I woman was referred for a fetal echocardiographic evaluation at 28 weeks of gestation because of the finding of hydrops fetalis on an obstetrical scan. The study demonstrated a large, complex, anterior mediastinal mass resulting in rotation of the heart leftward and posterior with compression of the right heart chambers (Figure 1⇓). The intracardiac anatomy was normal, with no significant inflow or outflow obstruction identified. Intrauterine removal of the mass was considered but declined by the parents. Preterm labor developed at 32 …