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.

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.

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.

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.

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.

Relation of brain natriuretic peptide to myocardial performance index in adults with congenital heart disease.

Myocardial performance index (MPI) is an echocardiographic Doppler-derived measure of ventricular function previously validated in patients with congenital heart disease. It may be preferred over conventional noninvasive measures of ventricular function in patients with complex anatomy because it is dependent on neither geometric shape nor heart rate. Brain natriuretic peptide (BNP) is a predictor of systolic and diastolic dysfunction in anatomically correct hearts. The correlation of BNP to MPI in patients with congenital heart disease was determined. Fifty-four adults with congenital heart disease were evaluated. BNP was measured using standardized assays. Doppler echocardiography was performed within 6 months of BNP assay. There were no changes in clinical status during this interval. An experienced observer was blinded and evaluated all echocardiographic images, and MPI and ejection fraction (EF) were determined. Left ventricular (LV) or univentricular MPI was calculated in 34 patients and right ventricular (RV) MPI was calculated in 23 patients. Pearson's correlation coefficient test showed that BNP significantly correlated with LV/univentricular MPI (r = 0.461, p = 0.006) and RV MPI (r = 0.748, p <0.0001), whereas LV/univentricular EF and RVEF had no significant correlation with BNP (r = -0.189, p = 0.172; r = 0.066, p = 0.729, respectively). In patients with congenital heart disease, BNP correlated significantly with MPI, but not with LV, RV, or univentricular EF. This is particularly true in patients with geometrically variable right ventricles in which EF may be more difficult to assess. In conclusion, these findings emphasize the unique ability of both BNP and MPI to assess global ventricular function in geometrically complex hearts.

Left ventricular apical wall motion abnormality is associated with lack of response to cardiac resynchronization therapy in patients with ischemic cardiomyopathy.

BACKGROUND: Many patients with appropriate indications fail to respond to cardiac resynchronization therapy (CRT). OBJECTIVE: The purpose of our study was to determine the relationship between CRT response and preimplantation apical wall motion abnormality. METHODS: We analyzed data from 83 patients with ischemic cardiomyopathy who underwent CRT. All patients had New York Heart Association class III or IV symptoms despite maximal medical therapy, left ventricular ejection fraction (LVEF) < or =35%, and QRS duration > or =130 ms or <130 ms with left ventricular dyssynchrony. CRT responders at 6 months were defined as surviving patients with: (1) no hospitalization for heart failure, and (2) improvement of New York Heart Association classification. Patients underwent echocardiography before and 6 months after implantation to assess changes in regional wall motion and LVEF. RESULTS: At baseline, CRT responders (n = 39) and nonresponders (n = 44) had similar LVEF (22.9% +/- 6.9% vs 23.1% +/- 8.3%), QRS duration (159 +/- 43 ms vs 159 +/- 36 ms), and medical treatment. CRT nonresponders had a higher prevalence of preimplantation apical wall motion abnormality (68% vs 33%, P = .003). Patients with baseline apical wall motion abnormalities (n = 43) were less likely than others (n = 40) to show improvement in wall motion at 6 months (30% vs 81%, P < .001) or clinical response to CRT (31% vs 64%, P = .003). CONCLUSION: The presence of a preimplantation apical wall motion abnormality was associated with a lower rate of CRT response in patients with ischemic cardiomyopathy.

Health-related quality of life: a closer look at related research in patients who have undergone the Fontan operation over the last decade.

The advancements in surgical technique and perioperative care have significantly improved the survival of children with single ventricle (SV) congenital heart disease (CHD) over the past decade. The population who have undergone the Fontan operation are growing into adulthood and facing many unique challenges. Past research has focused on functional and neurodevelopmental outcomes with inferences made to health-related quality of life (HRQOL). With the population who have undergone the Fontan operation surviving into adulthood, little research has been directed toward the self-report of HRQOL in adolescents and young adults after surgical palliation. Questions still remain on how these patients will transition into adulthood and whether they will live normal productive lives. This article reviews the literature related to HRQOL in the SV subgroup of CHD. In addition, an overview of newly developed disease-specific HRQOL instruments is presented as well as limitations and future research in HRQOL of the SV Fontan population.

Variations in adult congenital heart disease training in adult and pediatric cardiology fellowship programs.

OBJECTIVES: The purpose of this study was to evaluate adult congenital heart disease (CHD) training among U.S. cardiology fellowship programs. BACKGROUND: Although training recommendations for caring for adults with CHD exist, the educational patterns and numbers of specialists remain unknown. METHODS: We surveyed U.S. directors of 170 adult cardiology and 45 pediatric cardiology (PC) fellowship programs. Adult program surveys contained 1 single-response and 10 multiple-choice questions; pediatric program surveys contained 1 single-response and 13 multiple-choice questions. RESULTS: Ninety-four adult cardiology fellowship directors (55%) and 34 PC directors (76%) responded. Of adult programs, 70% were in university hospitals and 40% were associated with PC groups. Those with PC-affiliation had more adult CHD clinics (p < 0.02) and more adult CHD inpatient (p < 0.02) and outpatient (p < 0.002) visits than those without PC affiliation. Most PC programs were in children's hospitals (38%) or children's hospitals within adult hospitals (50%). Eighty-two percent had associated adult cardiology programs. Pediatric programs followed adult CHD patients in various care settings. Over one-third of adult and pediatric programs had < or = 3 lectures annually regarding adult CHD. Nine adult and 2 pediatric programs offered adult CHD fellowships, and only 31 adult and 11 pediatric fellows pursued advanced CHD training in the last 10 years. CONCLUSIONS: Adult CHD didactic and clinical experiences for cardiology fellows vary widely. Few programs offer advanced CHD training, and the number of specially trained physicians is unlikely to meet projected workforce requirements. Adult cardiology programs with PC affiliation have increased CHD experience and might provide good educational models.

Pathogenesis of thrombocytopenia in cyanotic congenital heart disease.

Although a significant minority of patients with cyanotic congenital heart disease (CCHD) are thrombocytopenic, the pathogenesis and prevalence have not been established. This study was designed to address these 2 issues. We included 105 patients with CCHD (60 men and 45 women; aged 21 to 54 years). Systemic arterial oxygen saturations were 69% to 78%. Hematocrits were 62% to 74% with normal iron indexes. In 26 of 105 patients (25%), platelet counts were <100x10(9)/L. The diagnosis was Eisenmenger syndrome in all 26 patients with thrombocytopenia. Platelet production was determined by flow cytometric reticulated platelet counts. Megakaryocyte mass was determined indirectly by thrombopoietin levels. Disseminated intravascular coagulation was based on prothrombin time, activated partial thromboplastin time, and D-dimers. Platelet activation was determined by levels of platelet factor 4 and beta thromboglobulin. Reference ranges were derived from 20 normal acyanotic controls. A reduction in absolute reticulated platelet counts implied decreased platelet production (p<0.001). Normal thrombopoietin levels implied normal megakaryocyte mass. Normal prothrombin time, activated partial thromboplastin time, and D-dimers excluded disseminated intravascular coagulation. Normal platelet factor 4 and beta thromboglobulin indicated absent or minimal platelet activation. Twenty-five percent of the patients with CCHD were thrombocytopenic because platelet production was decreased despite normal megakaryocyte mass. We hypothesized that right-to-left shunts deliver whole megakaryocytes into the system arterial circulation, bypassing the lungs where megakaryocytic cytoplasm is fragmented into platelets, thus reducing platelet production. In conclusion, platelet counts in CCHD appear to represent a continuum beginning with low normal counts and ending with thrombocytopenia.

Usefulness of post-ventriculotomy signal averaged electrocardiograms in congenital heart disease.

Signal-averaged electrocardiography is a sensitive and specific technique for detecting the slow conduction electrophysiologic substrates of reentrant monomorphic ventricular tachycardia. Although well established, the method has not been used for electrophysiologic assessment after right ventriculotomy for the intracardiac repair of congenital heart disease. This 8-year prospective study provided this assessment. Recordings were obtained from 242 post-ventriculotomy patients with congenital heart disease aged 16 to 72 years (139 males, 103 females). Because ventricular volume or pressure overload can prolong the QRS duration, 40 unoperated patients with hemodynamic overload served as controls. Orthogonal X, Y, and Z body surface electrodes were used to detect ventricular late potentials by permitting the examination of portions of the electrocardiogram otherwise obscured by noise and artifacts. Positive signal-averaged electrocardiographic (SAECG) results were based on established criteria derived from 3 time-domain variables calculated by an automated algorithm. Late potentials were detected in 151 of 242 patients (62%) and were significantly higher than controls (p = 0.0001). Radiofrequency ablation of an inducible slow conduction substrate rendered 20 of 23 positive post-ventriculotomy SAECG results negative, and surgical revision of the ventriculotomy scar rendered 19 of 19 positive SAECG results negative. In conclusion, negative SAECG results connote the absence of a reentrant substrate, and therefore, the absence of risk for reentrant monomorphic ventricular tachycardia, whereas positive SAECG results connote the presence of a slow conduction substrate and the potential risk for monomorphic ventricular tachycardia.

Cyanotic congenital heart disease and coronary artery atherogenesis.

Hypoxemic erythrocytotic residents of high altitudes lack coronary atherosclerosis and have low cholesterol levels. It was postulated that hypoxemic erythrocytotic adults with cyanotic congenital heart disease (CCHD) might be analogous. The incidence of coronary atherosclerosis in this patient population has not been established, and hypocholesterolemia has not previously been recognized. Accordingly, 279 patients were divided into 4 groups: group A: 143 cyanotic patients not operated on (54 men and 89 women, aged 18 to 69 years); group B: 47 cyanotic patients (28 men and 19 women rendered acyanotic by operation at age 22 to 69 years); group C: 41 acyanotic patients not operated on (22 men and 19 women, aged 22 to 75 years); and group D: 48 patients acyanotic before and after operation (24 men and 24 women, aged 21 to 70 years). Coronary arteries were studied angiographically in 59 patients and at necropsy in 5 subjects aged 37 to 56 years. Total cholesterol was <160 mg/dl in 58% of group A, 52% of group B, 10% of group C, and 12% of group D (p <0.000001, chi-square analysis). Angiograms disclosed dilated coronary arteries without obstruction. Necropsy disclosed ectatic coronary arteries with structural abnormalities of the media. In conclusion, this study provides the first quantitative and qualitative data on antiatherogenic changes in lipoproteins in adults with CCHD. The coronary arteries are atheroma free because hypocholesterolemia acts in concert with the antiatherogenic properties of upregulated nitric oxide, hyperbilirubinemia, hypoxemia, and low platelet counts. The persistence of hypocholesterolemia after the surgical elimination of cyanosis suggests a genetic determinant.

Cardiac MR imaging and MR angiography for assessment of complex tetralogy of Fallot and pulmonary atresia.

Breath-hold electrocardiographically gated cardiac magnetic resonance (MR) imaging and contrast material-enhanced MR angiography are emerging as ideal techniques for the evaluation of complex congenital heart disease. Tetralogy of Fallot is the most common cause of cyanotic congenital heart disease and, in its classic form, is associated with varying degrees of hypoplasia of the central and peripheral pulmonary arteries, with valvar pulmonary atresia and collateral aortopulmonary vessels occupying the extreme end of the spectrum. Accurate assessment of the size and anatomy of the pulmonary arteries is often difficult with echocardiography and conventional cineangiography. Compared with echocardiography in particular, cardiac MR imaging with three-dimensional reconstruction has distinct advantages for pre- or postoperative assessment of pulmonary anatomy in patients with tetralogy of Fallot and pulmonary atresia. MR imaging enables the clear and complete depiction of anatomy and thus can provide additional information about pulmonary artery abnormalities that are difficult to evaluate with conventional cardiac imaging techniques.

Extramural coronary arteries in adults with cyanotic congenital heart disease.

Dilatation and tortuosity of extramural coronary arteries are prevalent in cyanotic congenital heart disease. Two pathogenetic variables are operative, namely endothelial vasodilator substances and medial structural abnormalities.

Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension.

Extrapulmonary and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension (PPH) and the clinical expressions that coincide with the radiologic features of these 2 forms of pulmonary hypertension have not previously been characterized. Computed tomography (CT) and pulmonary CT angiography (CTA) utilized GE High Speed Advantage or Siemens Evolution electron beam tomographic (EBT) scanners in 31 patients with cyanotic pulmonary vascular disease (PVD) (group A: 12 men and 19 women; mean age 38 +/- 4 years) and in 13 women with PPH-acyanotic PVD (group B: mean age 30 +/- 3 years). Ten group A and 3 group B patients underwent imaging twice, yielding 57 studies. Group A patients' extrapulmonary radiologic features included proximal pulmonary arteries that were consistently enlarged, and were aneurysmal in 13%, causing bronchial compression and atelectasis. Thromboses were uniformly present and mild in 71% and moderate to massive in 29% of patients. Massive proximal thromboses caused asphyxic death and augmented right-to-left shunts by increasing flow resistance. Mild to extensive mural calcific deposits occurred in 26% of patients. Intrapulmonary radiologic features included intrapulmonary embolic infarction, intrapulmonary hemorrhage, bronchial/systemic arterial collaterals, and neovascularity. Group B patients' extrapulmonary radiologic features included proximal pulmonary arteries that were consistently enlarged but not aneurysmal and devoid of thromboses. Mild to extensive mural calcific deposits occurred in 23% of these patients. Intrapulmonary radiologic features were confined to mosaic attenuation and bronchial/systemic collaterals.