Long-Term Outcomes After Atrial Switch Operation for Transposition of the Great Arteries.

BACKGROUND: For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes. OBJECTIVES: The authors aimed to determine factors associated with survival in a large cohort of such individuals. METHODS: This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS). RESULTS: From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) during a median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplantations, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 years vs 42.4 ± 6.8 years; P < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe right ventricle dysfunction based on echocardiography. CONCLUSIONS: For most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow. A simplified prediction score for 5-year adverse outcome is derived to help identify those at greatest risk.

Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes.

BACKGROUND: Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation. METHODS: A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described. RESULTS: Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1-50) months. CONCLUSIONS: HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.

Complex Determinants of Work Ability in Adults With Congenital Heart Disease and Implications for Clinical Practice.

BACKGROUND: "Work ability" is the employees' capacity to meet the demands of their job. As more patients with complex congenital heart disease (CHD) are now reaching adulthood, we assessed work ability and factors impacting livelihood in adult CHD. METHODS: The work ability index (WAI) questionnaire and patient health questionnaire-9 (PHQ-9) were administered at 2 Midwest adult CHD centres from February 2017 to 2018. RESULTS: Of the 267 participants (n = 157 males, 59%) with an average age of 35 ± 13 years, the majority (n = 204, 76%) were employed. Patients with complex CHD (n = 103, 39%) were less likely to have enrolled in college or completed a graduate degree (P = 0.0115), and more likely to have an annual income of < $50,000 (P = 0.0056) and lower WAI scores (P = 0.0026) than patients with simple and moderate CHD. Unemployed patients (n = 63, 24%) with complex CHD (n = 27, 43%) were more likely to have higher PHQ-9 scores (P = 0.0242) indicating mild, moderate, or severe depression (P = 0.0482) than unemployed patients with simple and moderate CHD. Patients with complex CHD had lower self-perception of work ability compared with patients with simple and moderate CHD (P = 0.0007). Finally, patients in NYHA Functional Class I had higher WAI scores than NYHA Class III-IV (P < 0.0001). CONCLUSIONS: This study demonstrates that employed patients with complex CHD have lower education level, income, and work ability. Unemployed patients are more likely to exhibit symptoms of depression and have low self-perception of work ability. Occupational health programs focusing on promoting general health perception, increasing exercise capacity, and improving psychosocial health must be considered to improve work ability in patients with adult CHD to maintain livelihood.

Management of Severe Coarctation of the Aorta During Pregnancy.

An 18-year-old primigravida woman underwent emergent percutaneous balloon stent repair of a severe coarctation of the aorta, allowing her to undergo an uneventful remaining pregnancy and the delivery of a healthy baby. Her case also demonstrates the association between maternal coarctation and fetal Shone complex. (Level of Difficulty: Beginner.).

Successful TPV Implantation in a Pregnant Patient With Right Ventricle to Pulmonary Artery Conduit Obstruction.

A patient with repaired double outlet right ventricle presented during early gestation with heart failure symptoms due to severe right ventricle-pulmonary artery conduit stenosis and insufficiency. In the first trimester, she underwent transcatheter therapy with Melody pulmonary valve implantation with excellent hemodynamic results and completed pregnancy without significant maternal complications. (Level of Difficulty: Advanced.).

Warden Procedure in a 77-Year-Old Man.

Partial anomalous pulmonary venous return is a rare congenital heart defect characterized by 1 or more but not all of the pulmonary veins draining somewhere other than the left atrium, thereby creating a left-to-right shunt. Over time, right-sided volume overload may develop with its subsequent complications. We present a case of isolated partial anomalous pulmonary venous return in an older patient who underwent a Warden procedure at age 77 years, with rapid improvement in right ventricular size and function.

Converting Fontan-Björk to 1.5- or 2-Ventricle Circulation.

Patients with tricuspid atresia and ventricular septal defect have in the past occasionally undergone a Fontan with "Björk" modification to create a connection between the right atrium and the right ventricular outflow tract. Although rarely performed now, patients with this physiology often face severe complications requiring reintervention. We hypothesize that surgical conversion to a 2-ventricle or 1.5-ventricle circulation can improve hemodynamics, clinical status, and thus increase time to transplant. We present 2 successful cases to illustrate this idea.

The long-term functional outcome in Mustard patients study: Another decade of follow-up.

OBJECTIVE: For over 20 years, we have followed a cohort of patients who underwent the Mustard procedure for d-transposition of the great arteries. The current study follows the same cohort from our last study in 2007 to reassess their functional capacity and quality of life. PARTICIPANTS: Of the original 45 patients, six patients have required cardiac transplant and 10 patients have died, including two of the transplanted patients. Twenty-five of the remaining patients agreed to participate in this current study. DESIGN: Patients underwent comparable testing to the previous studies when possible including exercise stress testing, echocardiography, MRI or CT evaluation of cardiac anatomy and function, Holter monitor, and quality of life questionnaire. RESULTS: Thirty-one percent of patients have experienced cardiac death either in the form of mortality or cardiac transplantation. The major cause of death was systemic right ventricular failure. Sixty-five percent have continuing abnormalities of rhythm. Exercise time and workload showed a statistically significant decrease from the original study (Time 1) to both 10-year (Time 2) and 20-year (Time 3) follow-up points. Right ventricular ejection fraction decreased significantly from the Time 1 to Time 2, and again to this current follow-up. Quality of life measures of energy level decreased significantly from the original study to both the Time 2 and Time 3. CONCLUSION: Cardiac mortality for Mustard patients remains high, and over time, systemic right ventricular ejection fraction, rhythm, exercise tolerance, and quality of life assessments show deterioration. There does not appear to be a single clear predictor of poor outcome.

Long Term Consequences of the Fontan Procedure and How to Manage Them.

In 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to treat most forms of functional single ventricles and has become the current standard of care for long-term palliation of all patients with single ventricle congenital heart disease. Since 1971, the Fontan procedure has undergone several variations. These patients require lifelong management including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function along with multi organ evaluation. As these patients enter middle age, there is increasing awareness regarding the long-term complications and mortality. This review highlights the long-term outcomes of the Fontan procedure and management of late sequelae.

Permanent nonselective His bundle pacing in an adult with L-transposition of the great arteries and complete AV block.

We report the placement of a permanent transvenous nonselective His bundle pacing lead in conjunction with a transvenous pacemaker/implantable cardioverter-defibrillator in an adult with Levo-Transposition of the Great Arteries (L-TGA) and a stenotic coronary sinus (CS) ostium, which would not accommodate a transvenous left ventricular (LV) pacing lead. Nonselective His bundle pacing provided a nearly identical ventricular activation pattern in this previously unpaced patient. Many L-TGA patients will have an eventual need for permanent pacing and, given the challenges of CS cannulation, His bundle pacing may represent a preferred modality rather than pure morphologic LV pacing or surgical systemic ventricular lead placement to achieve optimal electrical synchrony.

At the Heart of the Pregnancy: What Prenatal and Cardiovascular Genetic Counselors Need to Know about Maternal Heart Disease.

In the last decade, an increasing number of cardiac conditions have been shown to have a genetic basis. Cardiovascular genetic counseling has emerged as a subspecialty aiming to identify unaffected at-risk individuals. An important sector of this at-risk population also includes expectant mothers, in whom unique clinical challenges may arise. Genetic counselors, especially those in cardiovascular and prenatal settings, have an opportunity to identify and assist women who may benefit from cardiovascular care during pregnancy. This paper provides basic management and genetic evaluation principles for affected women, as well as guidance on identifying those who are at risk. We provide considerations for cardiac surveillance in pregnancy and the post-partum period. Finally, key psychosocial issues that appraise how to best provide support to at risk women as they make informed decisions are discussed. We propose that a team approach including cardiology, maternal fetal medicine, and genetic counseling best serves this patient population. Ongoing questions addressing an evidence based approach to cardiovascular genetic conditions in pregnancy still remain. Thus, well-designed research protocols are essential to mark progress in this area.

Molecular and Genetic Insights into Thoracic Aortic Dilation in Conotruncal Heart Defects.

Thoracic aortic dilation (AD) has commonly been described in conotruncal defects (CTDs), such as tetralogy of Fallot, double outlet right ventricle and transposition of the great arteries, and truncus arteriosus. Several theories for this have been devised, but fairly recent data indicate that there is likely an underlying histologic abnormality, similar to that seen in Marfan and other connective tissue disease. The majority of aortic dissection in the general population occurs after the age of 45 years, and there have been very few case reports of aortic dissection in CTD. Given advances in cardiac surgery and increasing survival over the past several decades, there has been rising concern that, as patients who have survived surgical correction of these defects age, there may be increased morbidity and mortality due to aortic dissection and aortic regurgitation. This review discusses the most recent developments in research into AD in CTD, including associated genetic mutations.

Multi-institutional evaluation of the indications and radiation dose of functional cardiovascular computed tomography (CCT) imaging in congenital heart disease.

Ventricular volumes and ejection fraction are often used in clinical decision making in patients with congenital heart disease (CHD). The referral diagnosis, radiation exposure and image quality of functional cardiac computed tomography (CT) in a relatively large cohort of patients of CHD has not been reported. This is a retrospective evaluation of functional CT studies performed in CHD patients from three institutions (1/2007-3/2013). Patient and scanner characteristics, radiation dose estimates and image quality were compared. Two hundred ninety-eight functional CT studies were evaluated. The most common referral diagnosis were tetralogy of Fallot (33 %), transposition complexes (24 %) single ventricle heart disease (15 %), and left sided obstruction (15 %). The reason for cardiac CT was presence of pacemaker (60 %), need for detailed coronary artery imaging (18 %), metallic artifact in CMR (12 %), evaluation of prosthetic valve function (4 %), and claustrophobia or BMI too large for the available MR scanner (6 %). 266 (89.3 %) scans allowed quantification of ventricular function, 25 (8.4 %) scans allowed qualitative assessment of function, and 7 (2.3 %) of the scans were non-diagnostic for functional analysis. Median DLP was 399 mGy cm (186, 614), and median effective dose was 5.5 mSv (2.6, 8.5). Radiation dose and image quality varied across institutions. Cardiac CT function imaging can be performed in patients with congenital heart disease when CMR is contraindicated or has poor image quality. Radiation dose and image quality varies across institutions.

Evaluation by MRA of aortic dilation late after repair of tetralogy of Fallot.

OBJECTIVES: This study evaluated predictors for aortic dilation (AD) in patients with repaired tetralogy of Fallot (rTOF) using magnetic resonance angiography (MRA). BACKGROUND: AD is common in patients with rTOF and may result in increased morbidity and mortality. There are no guidelines for evaluation of AD for rTOF patients. METHODS: All adults with rTOF who previously underwent MRA had retrospective aortic measurements at the sinuses of Valsalva (SoV) and ascending aorta (AsAo). Rate of change in diameter was determined in patients with multiple MRAs. Chart review identified risk factors for AD. Univariate and multivariate analyses tested predictors of AD. RESULTS: Of the 87 patients who met the inclusion criteria, 12 (14%) had AD. At baseline, mean diameter was 3.6 ± 0.6 cm and 3.1 ± 0.6 cm at the SoV and AsAo, respectively. The AsAo was larger than the SoV in 17%. Predictors of AD included male gender, age, right aortic arch, pregnancy, older age at complete repair, smoking, and systemic hypertension. Serial studies were available in 55 patients; the rate of growth was slow: 0.4 ± 0.9 mm/year (SoV) and 0.1 ± 0.8mm/year (AsAo). CONCLUSIONS: AD is common in rTOF at the SoV and AsAo. Transthoracic echocardiography, which does not always image the AsAo as well as MRA, may not image AD in rTOF in cases in which the AsAo is dilated. Although several risk factors correlate with AD in rTOF, the rate of aortic growth is slow, suggesting that rTOF patients may not require frequent aortic imaging.