Echocardiographic differences between D-TGA and L-TGA in adult patients.

Transposition of the great arteries (TGA) is a common cardiac malformation in which the great arteries are discordant relative to the ventricles. The two common forms of transposition include D-TGA, which presents with cyanosis early in life, and L-TGA, which on the other hand, may permit survival to adulthood without being diagnosed in childhood. There are remarkable differences between these two forms of TGA in the clinical presentation, echocardiographic findings, and long-term outcomes. Multimodality imaging in patients with TGA usually provides diagnostic and hemodynamic assessment for routine follow-up and preoperative planning prior to surgical or transcatheter intervention. In this review, we present a summary of the fundamental echocardiographic aspects of these two forms of TGA with emphasis in the adult congenital heart disease population.

A practical guide to echocardiographic evaluation of adult Fontan patients.

Recent advances in surgical techniques and perioperative care for patients with single ventricle physiology have led to a remarkable improvement in long-term survival, such that now the majority of patients with single ventricle physiology are living to adulthood after Fontan palliation. The management of adult patients with Fontan physiology is one of the most challenging clinical dilemmas encountered in contemporary cardiology. The complex and heterogeneous anatomical and physiological abnormalities seen in Fontan patients mandate that any clinical evaluation, either for routine follow-up or preoperative evaluation prior to any transcatheter or surgical intervention, incorporates detailed information from a careful and thorough echocardiographic examination, These examinations, however, can be complex and confusing, even for experienced echocardiographers. Ideally, the interpretation of these studies is informed by an understanding of the basic anatomical lesions and of the potential long-term complications encountered in adult single ventricle patients. In this review, we present a practical and clinically oriented approach to the echocardiographic evaluation of adult patients with single ventricle physiology post-Fontan.

Echocardiography in adult patients with PDA: A simplified approach.

Patent ductus arteriosus is a common congenital heart anomaly in the newborn and children. It is often diagnosed and treated in childhood; however, there are a subset of patients who survive to adulthood undiagnosed, and it is present in adulthood with various clinical presentations. The clinical presentation, hemodynamics, and management of PDA presentation in adults depend on various factors, primarily the size of PDA, magnitude of shunting, and status of pulmonary vasculature. Echocardiography is often the primary imaging modality in the evaluation of adult patients with PDA and is an important tool that provides diagnostic and hemodynamic assessment for the initial evaluation and routine follow-up after PDA closure. In this review, we present a simplified approach of basic echocardiographic assessment of various types of PDA presentations in adults.

Patent foramen ovale and atrial septal defect.

Atrial septal defects (ASD) are among the most common congenital heart diseases encountered in adulthood. Patent foramen ovale (PFO) is present in up to 25% of the population. ASD could present as isolated lesion or in association with more complex congenital heart disease form as tetralogy of Fallot, or Ebstein's anomaly of tricuspid valve. There is a wide range of clinical presentation ranging from asymptomatic subjects surviving to adulthood undiagnosed to subjects presenting with right heart failure and severe pulmonary vascular disease (Eisenmenger syndrome). This manuscript is an in depth review of the complex atrial septation, the variable clinical presentation of ASD and PFO, and its clinical and therapeutic implications.

Reversal of severe mitral regurgitation by device closure of a large patent ductus arteriosus in a premature infant.

We report a critically ill premature infant with severe mitral valve regurgitation associated with pulmonary hypertension and a severely dilated left atrium from a large patent ductus arteriosus. The mitral valve regurgitation improved significantly with normalisation of left atrial size 4 weeks after percutaneous closure of the patent ductus arteriosus. This case highlights the potential reversibility of severe mitral valve regurgitation with treatment of an underlying cardiac shunt.

Left main coronary artery embolization in an 11-year-old girl due to inflammatory myofibroblastic tumor of the mitral valve.

This report describes a rare case of a subtotal left main coronary artery occlusion from mitral valve tumor embolization in an 11-year-old African American girl. This case is the first to report isolated ST segment elevation in lead aVR as a sign of a subtotal left main coronary artery occlusion in the pediatric population. In our case, we report a rare case of inflammatory myofibroblastic tumor of the mitral valve presenting with acute myocardial infarction due to embolization into the left main coronary artery. Coronary intervention was successfully performed using an aspiration catheter. Inflammatory myofibroblastic tumor usually presents as a solitary pulmonary nodule. Intracardiac involvement has been rarely reported. Despite the benign nature of the tumor, fatal presentations can occur. Early recognition and rapid intervention can be lifesaving in these patients.

Impact of Transcatheter Intervention on Myocardial Deformation in Patients with Coarctation of the Aorta.

Myocardial deformation measured by speckle tracking echocardiography can detect subtle abnormalities of left ventricular function before an obvious abnormality in traditional echocardiographic parameters such as ejection fraction is seen. We hypothesized that patients with coarctation of the aorta (CoA) may have impaired myocardial deformation that may persist even after successful transcatheter intervention. This is a retrospective study to assess the myocardial deformation in patients undergoing transcatheter intervention of CoA. The data were compared with age-matched normal controls. Echocardiographic parameters were obtained before, immediately and at median 6 months (range 3-8 months) after transcatheter intervention. Myocardial deformation indices were obtained off-line using Tomtec 2D Cardiac Performance Analysis Software. Repeated measure ANOVA was used to compare the indices between three time points. Independent sample t test or Chi-square test was used to compare data between groups. Twenty-four patients (age 13.5 ± 7.7 years) underwent successful transcatheter CoA intervention (stent 19, balloon angioplasty five) improving CoA peak gradient in the catheterization laboratory from 27.1 ± 11.3 to 4.5 ± 3.0 mmHg (p < 0.001). To compare with normal controls (n = 25, age 14.5 ± 1.9 years), fraction shortening was significantly higher in pre-intervention CoA patients (40.4 ± 7.1 vs. 33.8 ± 2.4 %, p < 0.001). In contrast, CoA patients had significantly abnormal left ventricular longitudinal strain compared to normal controls (-14.9 ± 2.6 vs. -20.5 ± 1.8 %, p < 0.001). In CoA patients, left ventricular longitudinal strain improved immediately and 3-8 months after intervention (-18.0 ± 2.9 and -17.6 ± 2.9 %, p < 0.007) but continued to be abnormal compared to normal controls. Patients with CoA had impaired myocardial deformation compared to normal controls. Myocardial mechanics improved but did not normalize even after successful transcatheter intervention on CoA in the short term.

Clinical Significance of Coronary Arterial Dominance: A Review of the Literature.

Coronary dominance describes the anatomic variation of coronary arterial supply, notably as it relates to perfusion of the inferior cardiac territories. Differences in the development and outcome in select disease states between coronary dominance patterns are increasingly recognized. In particular, observational studies have identified higher prevalence of poor outcomes in left coronary dominance in the setting of ischemic, conduction, and valvular disease. In this qualitative literature review, we summarize anatomic, physiologic, and clinical implications of differences in coronary dominance to highlight current understanding and gaps in the literature that should warrant further studies.

A case report of percutaneous MitraClip implantation in an adult with a double-outlet right ventricle.

Background: Atrioventricular valve regurgitation (AVVR) is present in up to 75% of Fontan patients, and it is associated with an increased risk of Fontan circulation failure, morbidity, and mortality. Traditional treatment options include surgical repair vs. surgical replacement. We present, to the best of our knowledge, one of the first cases of successful trans-catheter repair of severe common AVVR using the MitraClip device. Case summary: A 20-year-old male with a history of double-outlet right ventricle (DORV) with unbalanced common atrioventricular canal to the right ventricle, severely hypoplastic left ventricle, and total anomalous pulmonary venous return status post-Fontan procedure presented with progressively worsening dyspnoea on exertion. Transoesophageal echocardiogram demonstrated severe common AVVR. After discussion of the case during the adult congenital heart disease multidisciplinary conference, patient underwent successful placement of two MitraClip devices, reducing the regurgitation from torrential to moderate. Discussion: MitraClip therapy can be used to alleviate symptoms in patients deemed as high risk for surgery. However, careful attention must be paid to haemodynamics before and after clip placement, which may predict short-term clinical outcomes.

Myopericarditis in children: elevated troponin I level does not predict outcome.

Myopericarditis is primarily a pericarditic syndrome with some degree of myocardial involvement, as evident by elevated cardiac enzymes. Differentiating myopericarditis from acute coronary syndromes can be challenging and may require coronary angiography or perfusion studies. Data on myopericarditis and its outcome for children are scarce. This study delineates the demography, clinical presentation, and outcomes of myopericarditis for children and evaluates the prognostic value of elevated troponin I. The authors retrospectively reviewed 880 patients younger than 18 years of age who were admitted with diagnoses of chest pain, myocarditis, or pericarditis between 2000 and 2010 at their institution. Myopericarditis was defined as a clinical presentation of pericarditis in the presence of elevated levels of cardiac enzymes. Medical records were reviewed to abstract the demographic data, clinical presentation, evaluation, treatment, and follow-up outcomes. A total of 12 patients (1.4%) with myopericarditis were identified. All the patients were male, 8 (67%) of whom were Caucasian, and their median age was 16 years (range, 11-17 years). Two of the patients (17%) had recently used illicit drugs, and two (17%) had recently smoked cigarettes. At presentation, symptoms included chest pain in 12 patients (100%, 12/12), upper respiratory symptoms in 3 patients (25%, 3/12), and shortness of breath in 3 patients (25%, 3/12). No cardiac murmur or gallop was noted in any patient. Electrocardiographic (ECG) changes included diffuse ST-T changes (5 patients), localized ST-T changes (6 patients), and no ST-T changes (1 patient). All the patients had elevated levels of cardiac enzymes, with a median Troponin I level of 21.4 ng/ml (range, 5.0-134.4 ng/ml) and a median CK-MB level of 50.2 ng/ml (range, 7-135 ng/ml). Echocardiography showed normal left ventricular systolic function in all the patients (median ejection fraction, 61%; range, 56-69%). None had pericardial effusion during the first echocardiographic evaluation. Coronary angiography showed normal coronary arteries in all nine subjects for whom it was performed. Treatment of myopericarditis consisted of ibuprofen, acetaminophen, and/or aspirin. During a median follow-up period of 2 months (range, 2 weeks to 3 years), all the patients were asymptomatic with echocardiography showing normal left ventricular size and function. Myopericarditis was exclusively seen in male adolescents. Despite markedly elevated levels of cardiac enzymes, the clinical evolution of myopericarditis seems benign without any myocardial dysfunction. The inflammatory involvement of the myocardium appears to be self-limited without short-term, overt sequelae. An elevated troponin I level in myopericarditis, unlike acute coronary syndromes, does not seem to carry an adverse prognosis. Further studies are needed to evaluate the long-term prognosis for such patients.

Atherosclerotic Coronary Artery Disease in a Younger Adult With Transseptal Anomalous Left Coronary Artery.

We present a case of extensive coronary atherosclerotic disease in a younger patient with an anomalous left coronary artery with transseptal course and show the utility of multimodality evaluation to determine the culprit lesion in patients presenting with this rare association. (Level of Difficulty: Intermediate.).

Uhl's Anomaly With Left Ventricular Noncompaction: Role of Multimodality Imaging in a Rare Association.

Uhl's anomaly is a rare congenital heart disease characterized by partial or complete absence of the right ventricle myocardium. We report the first case, in a 21-year-old man, of Uhl's anomaly-associated left ventricular noncompaction. This association represents a unique clinical entity and has important implications for management strategies. (Level of Difficulty: Intermediate.).

A case report of right ventricular outflow tract obstruction caused by B-cell lymphoma: a rare presentation in an adult patient with pulmonary atresia.

BACKGROUND: Right ventricle outflow tract (RVOT) dysfunction is a common long-term complication in adult patients with pulmonary atresia/ventricular septal defect (PA/VSD). Common causes include valve thrombosis, stent fractures, and graft calcification. We present, to the best of our knowledge, the first case of malignant invasion of a Gore-Tex conduit, causing severe right ventricle (RV) failure. CASE SUMMARY: A 30-year-old woman with a history of PA/VSD with major aortopulmonary collateral arteries (MAPCAs) presented with worsening dyspnoea and exercise intolerance. In infancy, she underwent unifocalization of the right- and left-sided AP collaterals utilizing an 18 and 16 mm Gore-Tex graft, respectively. At age 7, she had surgical repair with VSD patch closure and placement of a 20 mm right ventricle-pulmonary artery (RVPA) homograft connected to a 20 mm Gore-Tex graft with linkage to the previously placed right and left unifocalization grafts. A transthoracic echocardiogram revealed a severely dilated RV and a heavily calcified RVOT conduit with severe stenosis. Cardiac computed tomography showed a stenotic RVPA conduit with calcified mural mass. She underwent surgical revision of the RVPA conduit with thromboendarterectomy of bilateral pulmonary arteries. Pathology of the removed conduit revealed fibrin-associated Epstein-Barr virus-positive diffuse large B-cell lymphoma (FA DLBCL). DISCUSSION: One prior case report has demonstrated invasion of DLBCL involving an aortic synthetic tube graft. However, malignant invasion of the RVOT Gore-Tex conduit has yet to be reported. Pathological review can be essential in guiding management. Malignant invasion of Gore-Tex conduits is a rare phenomenon, but one that should be closely monitored following repair of the RVOT.

Cardiac Rehabilitation for Adults and Adolescents With Congenital Heart Disease: EXTENDING BEYOND THE TYPICAL PATIENT POPULATION.

PURPOSE: Cardiac rehabilitation (CR) effectively decreases morbidity and mortality in adults after cardiovascular events. Cardiac rehabilitation has been underutilized for patients with congenital heart disease (CHD). The primary objective was to evaluate the inclusion of adolescents and adults with CHD in a CR program by analyzing data from our single-center CR program. The secondary objectives were to evaluate the efficacy and safety of CR as well as referral barriers. METHODS: This was a retrospective study of patients aged ≥15 yr who were referred to regional CR centers. Data on efficacy and safety were collected. RESULTS: Over a 4-yr period, 36 patients were referred to 23 regional centers: 23 patients completed CR, 12 are currently enrolled or in the referral process, and 1 died before initiation. The median age was 22 yr (range: 15-55). The primary indication was post-surgical (61%), followed by chronic heart failure (30%), and post-transplant (9%). After CR, metabolic equivalent tasks increased by 1.6 (P < .001), maximal heart rate increased by 13 beats/min (P = .026), exercise time increased by 1.35 min (P = .047), and treadmill speed increased by 0.7 mph (P = .007). There were no serious adverse events. All patients who completed CR remain alive at a median follow-up of 17 mo (range: 5-45). Common barriers to CR included accessibility, social circumstances, and cost for phase III CR. CONCLUSION: In our cohort, CR was effective and safe for adolescents and adults with CHD.

Computed tomography guided sizing for transcatheter pulmonary valve replacement.

OBJECTIVE: To evaluate the predictive value of Computed Tomography Angiography (CTA) measurements of the RVOT for transcatheter valve sizing. BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) provides an alternative to surgery in patients with right ventricular outflow tract (RVOT) dysfunction. We studied 18 patients who underwent catheterization for potential TPVR to determine whether CT imaging can be used to accurately predict implant size. METHODS: Cases were grouped by RVOT characteristics: native or transannular patch (n = 8), conduit (n = 5) or bioprosthetic valve (n = 5). TPVR was undertaken in 14/18 cases, after balloon-sizing was used to confirm suitability and select implant size. Retrospective CT measurements of the RVOT (circumference-derived (Dcirc) and area-derived (Darea) diameters) were obtained at the level of the annulus, bioprosthesis or conduit. Using manufacturer sizing guidance, a valve size was generated and a predicted valve category assigned: (1) <18 mm, (2) 18-20 mm, (3) 22-23 mm, (4) 26-29 mm and (5) >29 mm. Predicted and implanted valves were compared for inter-rater agreement using Cohen's kappa coefficient. RESULTS: The median age of patients was 37 years old (IQR: 30-49); 55% were male. Diagnoses included: Tetralogy of Fallot (12/18), d-Transposition repair (3/18), congenital pulmonary stenosis (2/18) and carcinoid heart disease (1/18). Measurements of Darea (κ = 0.697, p < 0.01) and Dcirc (κ = 0.540, p < 0.01) were good predictors of implanted valve size. When patients with RVOT conduits were excluded, the predictive accuracy improved for Darea (κ = 0.882, p < 0.01) and Dcirc (κ = 0.882, p < 0.01). CONCLUSIONS: CT measurement of the RVOT, using Darea or Dcirc, can predict prosthetic valve sizing in TPVR. These measurements are less predictive in patients with conduits, compared to those with a native RVOT or pulmonic bioprosthesis. CONDENSED ABSTRACT: We studied 18 patients who underwent catheterization for TPVR to determine whether CT imaging could be used to accurately predict implant size. Retrospective RVOT measurements were used to generate a predicted valve size, which was compared with implanted valve size for inter-rater agreement. Measurements of Darea (κ = 0.697, p < 0.01) and Dcirc (κ = 0.540, p < 0.01) were good predictors of implanted valve size. When cases with RVOT conduits were excluded, the predictive accuracy improved for Darea (κ = 0.882, p < 0.01) and Dcirc (κ = 0.882, p < 0.01). CT measurement of the RVOT can accurately predict prosthetic valve sizing in TPVR. These measurements are less predictive in patients with conduits.

Contraceptive Choices in the Immediate Postpartum Period in Women With Cardiac Disease.

Maternal cardiac disease (MCD) is associated with increased maternal and neonatal morbidity and mortality. Because unplanned pregnancies are especially risky, active use of reliable contraception is critical in this population. Studies in the noncardiac population have demonstrated that the postpartum period is an ideal time to address contraceptive plans. This retrospective cohort study was designed to describe contraceptive choices in women with MCD in the immediate postpartum period and to identify factors associated with specific contraceptive plans. We included women with MCD who delivered from January 2008 to September 2017 at a tertiary care institution with a multidisciplinary obstetrics and cardiology team. Maternal demographics, specifics of MCD, obstetrical outcomes, and contraceptive plans were obtained through chart review. Contraceptive plans were categorized into highly reliable methods (sterilization or long-acting reversible contraceptive methods) or less reliable methods (nonlong-acting reversible contraceptive methods or no contraception). In the 254 women included in this study, 40% planned to use highly reliable methods, while 60% planned to use less reliable methods. Women with cardiomyopathy were more likely to choose a highly reliable method of contraception (adjusted odds ratio 2.6, 95% confidence interval 1.2 to 5.7), a reassuring finding, given the particularly high risk of poor pregnancy outcome with this diagnosis. There were no differences in other cardiac diagnoses between the 2 contraceptive groups. In conclusion, the finding that <50% of postpartum women with MCD plan to use a highly reliable method of contraception warrants further examination to identify and address barriers to reliable contraceptive plans in this high-risk population.

Transcatheter pulmonary valve implantation: will it replace surgical pulmonary valve replacement?

INTRODUCTION: Right ventricular outflow tract (RVOT) dysfunction is a common hemodynamic challenge for adults with congenital heart disease (ACHD), including patients with repaired tetralogy of Fallot (TOF), truncus arteriosus (TA), and those who have undergone the Ross procedure for congenital aortic stenosis and the Rastelli repair for transposition of great vessels. Pulmonary valve replacement (PVR) has become one of the most common procedures performed for ACHD patients. Areas covered: Given the advances in transcatheter technology, we conducted a detailed review of the available studies addressing the indications for PVR, historical background, evolving technology, procedural aspects, and the future direction, with an emphasis on ACHD patients. Expert commentary: Transcatheter pulmonary valve implantation (TPVI) is widely accepted as an alternative to surgery to address RVOT dysfunction. However, current technology may not be able to adequately address a subset of patients with complex RVOT morphology. As the technology continues to evolve, new percutaneous valves will allow practitioners to apply the transcatheter approach in such patients. We expect that with the advancement in transcatheter technology, novel devices will be added to the TPVI armamentarium, making the transcatheter approach a feasible alternative for the majority of patients with RVOT dysfunction in the near future.