Echocardiographic evaluation of congenital left ventricular outflow obstruction.

Left ventricular outflow obstructions (LVOTO) encompass a series of stenotic lesions beginning within the anatomic left ventricular outflow tract and ending in the descending thoracic aorta, occurring alone or in series. The degree of stenosis and hemodynamic consequences may vary from mild and well tolerated to severe and functionally limiting. The majority of LVOTO (with the exception of calcific and rheumatic aortic stenosis) are congenital in etiology. Echocardiography has become an integral tool in the assessment of patients with congenital heart disease. This article is a contemporary review of the clinical features of LVOTO and the role of echocardiography in this patient population.

Usefulness of serum brain natriuretic peptide to predict adverse events in patients with the Eisenmenger syndrome.

The aim of this study was to evaluate the prognostic value of brain natriuretic peptide (BNP) in outpatients with the Eisenmenger syndrome (ES). BNP is often elevated in patients with cyanotic congenital heart disease. The clinical utility of BNP in patients with cyanotic congenital heart disease and the ES has not been clearly delineated. Records of adults with ES who had undergone serum BNP measurement were reviewed. The primary end point was death or heart failure admission. Fifty-three patients were included, with 15 patients (28%) meeting the primary end point (death in 7, heart failure hospitalization in 8). Mean and median baseline BNP in patients meeting the primary end point were 322 ± 346 and 179 pg/ml, compared to 100 ± 157 and 41 pg/ml in those not meeting the primary end point (p = 0.0029). A Cox proportional-hazards model using baseline BNP between the 2 groups yielded a hazard ratio of 1.84 (95% confidence interval [CI] 1.19 to 2.85, p = 0.006). The relative risk for baseline BNP level >140 pg/ml was 4.62 (95% CI 1.80 to 11.3, p = 0.008). Patients who met the primary end point increased their BNP levels by 42.5 pg/ml per year (95% CI 12.09 to 72.95, p = 0.006) compared to 7.2 pg/ml per year (95% CI 2.01 to 12.47, p = 0.007) in patients who did not meet the primary end point. In conclusion, elevated BNP levels are predictive of death or heart failure admission in patients with the ES. A serum BNP level >140 pg/ml is a useful tool in identifying high-risk patients.

Sex and age differences in body-image, self-esteem, and body mass index in adolescents and adults after single-ventricle palliation.

Single-ventricle congenital heart disease (SVCHD) requires multiple palliative surgical procedures that leave visible surgical scars and physical deficits, which can alter body-image and self-esteem. This study aimed to compare sex and age differences in body-image, self-esteem, and body mass index (BMI) in adolescents and adults with SVCHD after surgical palliation with those of a healthy control group. Using a comparative, cross-sectional design, 54 adolescent and adult (26 male and 28 female) patients, age 15­50 years, with SVCHD were compared with 66 age-matched healthy controls. Body-image and self-esteem were measured using the Multidimensional Body-Self Relations Questionnaire­Appearance Scale and Rosenberg Self-Esteem Scale. Height and weight were collected from retrospective chart review, and BMI was calculated. Female adolescents and adult patients with SVCHD reported lower body image compared with males patients with SVCHD and healthy controls (p = 0.003). Specific areas of concern were face (p = 0.002), upper torso or chest (p = 0.002), and muscle tone (p = 0.001). Patients with SVCHD who were \21 years of age had lower body image compared with healthy controls (p = 0.006). Self-esteem was comparable for both patients with SVCHD and healthy peers. There were no sex differences in BMI; BMI was higher in subjects[21 years of age (p = 0.01). Despite the similarities observed in self-esteem between the two groups, female patients with SVCHD\21 years of age reported lower perceived body-image. Our findings support the need to recognize poor psychological adjustment related to low self-esteem in patients with SVCHD; female patients warrant increased scrutiny. Strategies to help patients with SVCHD cope with nonmodifiable aspects of body-image during the difficult adolescent­to­young adult years may potentially enhance self-esteem and decrease psychological distress.

Quality of life, health status, and depression: comparison between adolescents and adults after the Fontan procedure with healthy counterparts.

BACKGROUND: Quality of life (QOL) in adolescents and adults who have undergone the Fontan procedure and are living with only 1 ventricle is presumed to be diminished. OBJECTIVES: This study aimed to compare QOL, health status, and prevalence of depression in adolescents/adults after the Fontan procedure with healthy counterparts and to identify predictors of QOL in the Fontan group. METHODS: Using a comparative, cross-sectional design, 54 adolescents and adults with single ventricle congenital heart disease who have undergone the Fontan procedure were compared with 66 age-matched healthy counterparts. Quality of life, health status, depression, and social support were measured using the Satisfaction With Life Scale, Short Form Survey Version 2, Patient Health Questionnaire Depression Module, and Multidimensional Scale of Perceived Social Support. Clinical variables were abstracted from medical records. Predictors of QOL were determined using multiple linear regression. RESULTS: Adolescents and adults in the Fontan group reported lower physical health status (mean [SD] = 46.5 [9.3] vs mean [SD] = 55.9 [5.1], P < .001) and were more depressed (mean [SD] = 7.3 [5.9] vs mean [SD] = 4.5 [4.3], P < .004) than their healthy counterparts. There were no differences in QOL, mental health status, or social support between the 2 groups. Functional status (New York Heart Association class), depression, and social support accounted for 55% of the variance in QOL in the Fontan group. CONCLUSIONS: Despite lower levels of physical health, the QOL of Fontan patients was comparable with that of their healthy counterparts; this finding contradicts previous proxy reports, self-reports, and assumptions that QOL is lower in patients with complex single ventricle congenital heart disease. However, because Fontan patients were more depressed than their healthy counterparts, the need for early screening and detection is warranted.

Common congenital heart disorders in adults: percutaneous therapeutic procedures.

The population of adults with unrepaired, palliated, and repaired congenital heart disease is growing. Surgical intervention, once the mainstay of palliative and corrective therapy, has been supplanted in many instances by less invasive trans-catheter techniques. Hybrid procedures involving the symbiotic use of surgical and trans-catheter techniques are increasingly utilized in patients with complex lesions. This article summarizes some of the currently applied trans-catheter interventional techniques for pulmonary stenosis, ASD, VSD, and coarctation of the aorta.

Patients with single-ventricle physiology: prognostic implications of stress testing.

This study used a retrospective analysis of adults with single-ventricle physiology to ascertain the predictive power of cardiopulmonary stress-testing parameters in determining patients at increased risk of suffering from adverse clinical outcomes. We found that the specific parameters of percent of maximum predicted heart rate achieved and maximum oxygen consumption were significantly correlated with adverse clinical outcomes in patients with single-ventricle congenital heart disease.

Relationship between mechanical and electrical remodelling in patients with cardiac resynchronization implanted defibrillators.

AIMS: Cardiac resynchronization therapy (CRT) is associated with reverse left ventricular (LV) remodelling. However, the effects of CRT-induced mechanical remodelling on electrical remodelling, and the occurrence of ventricular arrhythmias have not been clearly established. We studied the relationship between mechanical remodelling, electrical remodelling, and the occurrence of appropriate implantable cardioverter-defibrillator (ICD) therapy 1 year after CRT. METHODS AND RESULTS: We analysed data from 45 patients who underwent ICD-CRT implantation at our centre. Significant LV reverse remodelling was defined by a minimum 10% decrease in the LV end-diastolic diameter (LVEDd) at 1 year of follow-up. Electrocardiographic indices of dispersion of repolarization [QTc, Tpeak-Tend (Tp-e) and their dispersion] were measured immediately and 1 year post-CRT implantation. The occurrence of appropriate ICD therapy was noted for each patient. Patients with (n= 21) and without (n= 24) significant LV reverse remodelling had similar baseline characteristics. At 1 year of follow-up, patients with mechanical reverse LV remodelling exhibited a significant decrease in QTc (505 ± 42 vs. 485 ± 52 ms, P < 0.05) and Tp-e (107 ± 26 vs. 92 ± 22 ms, P < 0.0001). However, patients without mechanical LV reverse remodelling exhibited a significant increase in QT dispersion (29 ± 43 vs. 98 ± 47 ms, P = 0.002) and Tp-e dispersion (22 ± 21 vs. 54 ± 36 ms, P = 0.0001). Finally patients with mechanical LV reverse remodelling experienced a lower rate of ICD therapy (P = 0.0025) after a mean follow-up of 19 months. CONCLUSION: Reverse LV mechanical remodelling is associated with reversal of electrical remodelling and a lower rate of appropriate ICD therapy following CRT.

Effects of pulmonary vasodilator therapy on ventilatory efficiency during exercise in adults with Eisenmenger syndrome.

OBJECTIVE: Eisenmenger syndrome, characterized by systemic-level pulmonary arterial resistance with resultant right-to-left shunt, is associated with low exercise capacity and hyperpnea at rest and exercise. Because ventilatory requirements are augmented by right-to-left shunting, we hypothesized that if pulmonary vasodilator treatment improved pulmonary perfusion in this condition, this would also improve ventilatory efficiency during exercise. DESIGN: To investigate this, data from incremental cardiopulmonary exercise tests performed by Eisenmenger patients before and after beginning therapy with pulmonary hypertension medications were retrospectively analyzed. Setting. Ahmanson/University of California, Los Angeles Adult Congenital Heart Disease Center. PATIENTS: Ten adults with Eisenmenger syndrome treated with either bosentan or sildenafil. OUTCOME MEASURES: The primary analysis was comparison, before and after treatment, of the efficiency of exercise ventilation as reflected in the ratio of ventilation (.V(E)) to carbon dioxide output (.VCO2) measured at the anaerobic threshold (AT), the slope of .V(E)/.VCO2 during incremental exercise, and end tidal partial pressure of CO2 (PETCO2) at the AT. Secondary measures included peak oxygen uptake (.VO2) and AT. RESULTS: Following treatment there were significant reductions in the slope .V(E)/.VCO2 (59.5 ± 12.9 vs. 50.0 ± 7.2, P= .003), and significant decrease in .V(E)/.VCO2 ratio (56.9 ± 6.2 vs. 50.2 ± 5.9, P= .00004) and increase in PETCO2 (21.12 ± 2.43 vs. 23.9 ± 2.62 torr, P= .0092) measured at the AT. Increases in peak .VO2 (0.73 ± 0.25 vs. 0.78 ± 0.32 L/min, P= .333) and AT (0.61 ± 0.20 vs. 0.68 ± 0.25 L/min, P= .154) were not significant. CONCLUSIONS: These findings are consistent with reduction in right-to-left shunt due to improved pulmonary blood flow, though attenuation of ventilatory drive is not excluded. Treatment of adult Eisenmenger patients with pulmonary the pulmonary vasodilators bosentan or sildenafil leads to improvement in parameters of ventilatory efficiency during exercise.

Clinical profile of the adolescent/adult Fontan survivor.

OBJECTIVES: The study aims to describe the clinical profile of the adult Fontan survivor and identify the worries, symptoms, and the impact of cardiac surveillance most commonly experienced. DESIGN: A descriptive, cross-sectional design was used. SETTING: The study was performed in outpatient adult and pediatric cardiology clinics in university-affiliated and private practice offices. PATIENTS: Fifty-four adolescent and adult patients with single ventricle congenital heart disease who have undergone the Fontan procedure participated in the study. The mean age was 26 ± 9 years with 52% female and 63% Caucasian. OUTCOME MEASURES: Demographic and clinical data were obtained by a standard intake form and retrospective chart reviews. The Congenital Heart Disease TNO/AZL Adult Quality Of Life questionnaire was completed to assess worries, symptoms, and the impact of cardiac surveillance. RESULTS: The majority were single (73%), employed or full-time students (93%), with health insurance (94%), had a single left ventricle (78%), the diagnosis of tricuspid atresia or double inlet left ventricle (59%), lateral tunnel Fontan type (44%), history of arrhythmias (76%), left ventricle ejection fraction percentage >50 (66%), oxygen saturations >90% (70%), frequent headaches (50%), scoliosis (22%), varicose veins, ascites, and liver cirrhosis (46%), normal body mass index (59%), and New York Heart Association class I (48%) and II-III (52%). Primary worries related to current health (83%), job/employment (69%), ability to work, (61%) and living independently (54%). The most bothersome symptoms were shortness of breath with ambulation (69%), dizziness (61%), and palpitations (61%). CONCLUSIONS: Fontan survivors experience residua and sequelae from multiple surgical procedures and the natural disease course. Our results support the need for ongoing assessment of both physical symptoms and psychosocial concerns, and suggest the need for multiple risk factor intervention strategies that improve physical and emotional health in Fontan survivors.

Two-dimensional and Doppler echocardiography reliably predict severe pulmonary regurgitation as quantified by cardiac magnetic resonance.

BACKGROUND: The grading of pulmonary regurgitation (PR) severity by two-dimensional (2D) and Doppler echocardiography is not standardized. Cardiovascular magnetic resonance imaging is the clinical gold standard for PR quantification. The purpose of this study was to determine the best 2D and Doppler echocardiographic predictors of severe PR. METHODS: Thirty-six patients with tetralogy of Fallot or pulmonary valve stenosis with prior pulmonary valvuloplasty or transannular or subannular patch repair underwent 2D and Doppler echocardiography and cardiovascular magnetic resonance. Two-dimensional and Doppler echocardiographic measurements used to predict severe PR included diastolic flow reversal in the main or branch pulmonary arteries, PR jet width > or = 50% of the pulmonary annulus, PR pressure half-time < 100 ms, and PR index < 0.77. RESULTS: With the exception of PR index, all indices were significant independent predictors of severe PR. The best univariate predictor of severe PR was branch pulmonary artery diastolic flow reversal. CONCLUSION: Two-dimensional and Doppler echocardiography reliably identified severe PR in this cohort.

Perventricular closure of a large ventricular septal defect in congenitally corrected transposition of the great arteries.

We report the case of a 30 year-old male with congenitally corrected transposition of the great arteries, atrial, and ventricular septal defects (VSD), and pulmonary stenosis. He previously underwent three palliative surgical procedures before undergoing intracardiac repair at age 20 with a left ventricular to pulmonary artery (LV-PA) conduit, VSD closure, and replacement of the systemic atrioventricular valve. A residual VSD was noted postoperatively. He did well for approximately 10 years when he started becoming more breathless with daily activities and was noted to have a resting room air oxygen saturation of 85%. Despite increased diuretic therapy he continued to deteriorate and was ultimately admitted to the hospital in florid right and left heart failure with recurrent atrial fibrillation. Catheterization revealed pulmonary hypertension (pulmonary artery pressure = 80/17 mm Hg), moderate conduit stenosis, severe pulmonic regurgitation, and oxygen saturation of 75%. Calculated shunt fraction (Qp : Qs) was 1.3:1. He was referred for surgical intervention, specifically, LV-PA conduit replacement, oversewing of the pulmonic valve, VSD closure, and pacemaker placement. Intraoperatively, the VSD could not be closed despite multiple attempts through various approaches. Therefore, perventricular VSD closure using two Amplatzer septal occluders (AGA Medical, Golden Valley, MN) was performed in the operating room with the chest open off cardiopulmonary bypass. Following deployment, the residual shunt was small and the inferior vena cava-to-pulmonary artery saturation step-up was only 4%. The left ventricular systolic pressure decreased to one half systemic. This case highlights the utility and efficacy of a hybrid approach in the treatment of complex congenital heart disease.

A blue baby grows up.

This report describes the history of a 41-year-old female patient born with atresia of the right atrioventricular valve, hypoplasia of the right ventricle, an atrial septal defect, and malposed great arteries. The patient underwent surgical pulmonary artery banding in infancy but did not undergo corrective surgery due to the development of pulmonary arterial hypertension. This report touches on several important clinical issues that may arise when caring for the adult with complex palliated cyanotic congenital heart disease, including the development and treatment of pulmonary arterial hypertension.

Isovolumic cardiac contraction on high-temporal-resolution cine MR images: study in heart failure patients and healthy volunteers.

PURPOSE: To prospectively implement high-temporal-resolution cine magnetic resonance (MR) imaging protocol to compare cardiac preejection contraction (PEC) and prefilling relaxation (PFR) times between heart failure (HF) patients and healthy control subjects and to assess accuracy of PEC times to stratify HF patients, with ejection fraction (EF) and New York Heart Association (NYHA) symptom class as reference standards. MATERIALS AND METHODS: Following institutional review board approval of this HIPAA-compliant study and written informed consent, 18 healthy volunteers (10 women, eight men; mean age, 43 years +/- 14 [standard deviation]) and 18 HF patients (five women, 13 men; mean age, 49.8 years +/- 3) were imaged (breath-hold true fast imaging with steady-state precession, with temporal resolution of 5.6 msec at 1.5 T). By using left ventricular (LV) outflow tract acquisition, PEC phase was defined as time at QRS trigger to immediately before aortic valve opening. PFR was defined as time from initial aortic valve closure to immediately before mitral valve opening. Group means were compared (unpaired Student t test). Accuracy of PEC parameters in stratifying participants with severe systolic HF on the basis of EF and NYHA symptom class was assessed (receiver operating characteristic curve analysis). RESULTS: Compared with control subjects, HF patients had prolonged mean PEC time (40.4 msec +/- 11.8 vs 91.3 msec +/- 26, P < .001) and mean PFR time (68.3 msec +/- 26.8 vs 103.7 msec +/- 41.8, P < .01). PEC time correlated with global EF (r = -0.73, P < .001) and LV mass (r = 0.69, P < .001). For identification of patients with severe LV systolic dysfunction (EF 2), PEC time had good accuracy (AUC, 0.875 [P < .001]). CONCLUSION: It is feasible to assess isovolumic PEC and PFR phases of the cardiac cycle with high-frame-rate cine MR images, and PEC time is a surrogate measure of moderate-to-severe systolic HF.

Prevalence of migraine headaches in patients with congenital heart disease.

The prevalence of migraine headaches (MH) is 12% in the general population and increases to 40% in patients with patent foramen ovale. This study evaluated the prevalence of MH in patients with congenital heart disease (CHD). Of 466 patients contacted from the UCLA Adult Congenital Heart Disease Center, 395 (85%) completed a questionnaire to determine the prevalence of MH. Patients were stratified by diagnosis of right-to-left, left-to-right, or no shunt. A group of 252 sex-matched patients with acquired cardiovascular disease served as controls. The prevalence of MH was 45% in adults with CHD compared to 11% in the controls (p<0.001). Of the 179 patients with MH, 143 (80%) had migraines with aura and 36 (20%) had migraines without aura versus 36% and 64% observed in the controls (p<0.001). The frequency of MH was 52% in the right-to-left shunt group, 44% in the left-to-right, and 38% in the no shunt group (p=NS). In patients with a right-to-left shunt who underwent surgical repair, 47% had complete resolution of MH, whereas 76% experienced >50% reduction in headache days per month. In conclusion, the prevalence of MH in all groups of adults with CHD is 3 to 4 times more than a sex-matched control population, with increasing prevalence of MH in patients with no shunt, left-to-right, and right-to-left shunt. The higher than expected frequency of MH in patients with CHD without an intracardiac shunt, suggests additional mechanisms to explain the significant association with MH.