An adolescent with chest pain and cardiac hemangioma.

Less than 5% of chest pain in children is cardiac in origin, yet this complaint still represents one of the top reasons children are referred to pediatric cardiologists. This article describes a patient whose cardiac tumor illustrates the challenges of evaluating pediatric chest pain and the Standardized Clinical Assessment and Management Plan algorithm that can help.

Quantitation of fetal heart function with tissue Doppler velocity imaging-reference values for color tissue Doppler velocities and comparison with pulsed wave tissue Doppler velocities.

Quantitative assessment of fetal heart function has been difficult. Increasingly, tissue Doppler imaging (TDI) is used to measure fetal cardiac function noninvasively. There are two principal techniques, spectral pulsed wave (PW) TDI and color TDI (CTDI). Published reference values for fetal myocardial velocities are based on spectral PW TDI only. However, previous phantom, adult, and animal studies have shown that PW TDI velocities are systematically higher than CTDI velocities. There are no fetal studies so far. We hypothesized that myocardial velocities derived by PW TDI and CTDI are significantly different in the fetus. This prospective observational study included 91 fetuses (gestational age 28.6 ± 6.6 weeks; range 19-40 weeks) seen for routine prenatal ultrasound. From apical 4-chamber views, tricuspid ring (right ventricle), lateral and septal mitral ring were sampled by PW TDI and CTDI. Bland-Altman analysis was used for comparisons. PW and CTDI S' velocities correlated strongly in all three cardiac segments (r = 0.6 to 0.9; P < 0.01). There was a systematic bias toward higher velocities with PW TDI versus CTDI (bias 0.96 cm/s; 95% CI 1.08-0.85 cm/s). However, the strength of the correlation and bias varied depending on the region of the fetal heart sampled. PW TDI and CTDI velocity measurements are feasible in the fetus and correlate well. However, PW TDI velocities are higher than CTDI velocities with significant regional variation. This precludes a mathematical conversion of PW to CTDI in vivo. As PW TDI and color TDI vary, different reference values for fetal CTDI velocities were generated.

Right ventricular wall-motion changes after infant open heart surgery--a tissue Doppler study.

BACKGROUND: Right ventricular (RV) dysfunction is a well-recognized complication of cardiopulmonary bypass surgery (CPB) in adults. Infants and neonates may also be at high risk for this due to immature myocardium. Conventional assessment of RV function is just qualitative, but novel tissue Doppler echocardiographic (TDI) markers including peak systolic strain rate (SR) and isovolumic contraction acceleration (IVA) permit noninvasive quantitation of RV function. This study assessed myocardial velocities, IVA and SR in infants and neonates undergoing open heart surgery using TDI to study regional myocardial function perioperatively. METHODS: Transthoracic TDI data were obtained in the OR before and 24 hours post-CPB on 53 consecutive infants (age 0.39 ± 0.23 years). They were followed with TDI through hospital discharge. RESULTS: Mean CPB time was 87 ± 49 min (cross-clamp 52 ± 26 min). Peak systolic (STDI ) and diastolic myocardial velocities (ETDI , ATDI ), IVA, and peak SR were recorded in RV and LV from standard views for offline analysis. Postoperatively, LV systolic function and diastolic longitudinal function were unchanged or improved from baseline. LV radial velocities were increased postoperatively indicating adequate support. In contrast, RV longitudinal systolic and diastolic function was significantly diminished after CPB. RV changes persisted through hospital discharge. CONCLUSIONS: In infants and neonates, perioperative measurements of systolic and diastolic tissue Doppler parameters are feasible and revealed significant RV systolic and diastolic dysfunction post-CPB with preserved LV function. As such, TDI provides a sensitive tool to monitor the infant heart after CPB and may potentially be useful to assess different myocardial protection strategies.

Increased left main coronary artery dimensions in children with sickle cell disease.

For adults with sickle cell disease (SCD), cardiac changes are well described and may account for up to one-fourth of disease-related mortality. Much less is known about heart disease in pediatric SCD patients. This retrospective study analyzed a contemporary cohort of children with SCD followed at a single tertiary care center. The study aimed to evaluate the effect of SCD on left main coronary artery (LMCA) dimensions in the contemporary pediatric population. The echocardiography database was searched for studies of children with SCD from 2000 to 2009. The LMCA diameter was remeasured offline from digital images. Digital echocardiographic studies of 68 children (35 boys) 11.7 ± 4.6 years of age with SCD were analyzed. All the patients had normal systolic function as measured by fractional shortening. The left ventricular end-diastolic dimension (LVEDD) for 34 % of the pediatric SCD patients showed a dilated LV. In 24 % of the children, a LV mass index (LVMI) greater than 50 g/m(2.7) denoted LV hypertrophy. Application of sex-specific normal values showed that 60 % of the boys and 33 % of the girls had an increased LVMI. The LMCA diameter was enlarged in 43 % of the SCD children, including 26 % with an LMCA z-score higher than 3. This study found a higher incidence of LMCA dilation in a pediatric referral population with SCD. The findings also validated an increased LVMI in the contemporary SCD patient population, which was noted previously. This study adds the need to include assessment of coronary arteries in cardiac evaluation of SCD patients, and special attention should be paid to patients with a high-normal to high coronary z-score.

Angina pectoris with troponin increase in arrhythmogenic right ventricle dysplasia: case article and review of the literature.

A 16-year-old Hispanic girl with arrhythmogenic right-ventricle dysplasia (ARVD) presented with angina pectoris and troponin increase on three occasions. There was a family history of sudden cardiac death in a cousin. Her mother was diagnosed with ARVD. The patient herself had a history of nonsustained ventricular tachycardia but did not meet diagnostic criteria for ARVD. Cardiac workup, including serial transthoracic echocardiograms and a coronary angiogram, showed a structurally normal heart without coronary artery stenosis. Results of cardiac magnetic resonance imaging were questionable, but endomyocardial biopsy did not show evidence of viral myocarditis by polymerase chain reaction. Genetic testing confirmed ARVD.

Exercise stress echocardiography after childhood Ross surgery: functional outcome in 26 patients from a single institution.

Adult studies suggest a better functional outcome after aortic valve replacement with a pulmonary autograft compared with mechanical or homograft valves. Little is known about functional results after Ross surgery in growing children. This study reports formal exercise stress echocardiographic data from 26 pediatric Ross patients. A retrospective cohort study analyzed stress echocardiographic data of patients who underwent Ross surgery as a child (<17 years old). All patients were operated by a single surgeon and underwent a Bruce protocol stress echocardiogram on the treadmill. Twenty-six patients (4 girls) were 9.3 ± 5.0 years at surgery and 14.9 ± 3.5 years (range 6.6-19.7 years) at follow-up. Mean follow-up was 5.4 ± 3.7 years (median 4.2). All were asymptomatic. The exercise time was normal in 87% of cases at 12.8 ± 2.5 min. On stress echocardiography, the mean right-ventricular outflow tract (RVOT) gradient increased from 38 ± 22 mmHg at rest to 82 ± 33 mmHg after exercise, but this did not correlate with exercise times. Stress echocardiography is useful in evaluating patients after childhood Ross surgery for aortic valve disease. In this pediatric cohort, most patients achieved normal exercise capacity. The presence of mild or moderate RVOT obstruction had no significant impact on exercise capacity.

Clinical impact of the baseline echocardiogram in children with high-risk acute lymphoblastic leukemia.

BACKGROUND: It is common practice to hold anthracycline induction chemotherapy in children with high-risk acute lymphoblastic leukemia (HR-ALL) until an echocardiogram is performed and interpreted. It is unclear whether withholding therapy in HR-ALL children is justified by echocardiogram findings. We reviewed the initial echocardiograms in a cohort of children with HR-ALL to determine the incidence of contraindications for anthracycline treatment. PROCEDURE: We identified 50 consecutive children (<21 years old) with HR-ALL presenting at our institution over a 10-year period. One didn't have an initial echocardiogram, 39 had pre-therapy studies, and 10 were studied within 6 days of beginning chemotherapy. These 49 studies were reviewed to determine the incidence and clinical significance of abnormalities. RESULTS: All 49 patients had normal cardiac function. Initial echocardiogram findings had no impact on induction chemotherapy administration in any patient. However, only 22(45%) of the studies were completely normal. Echocardiographic abnormalities included pericardial effusion (17/49), trivial or mild mitral or aortic insufficiency (13/49), left ventricular enlargement (3/49), and structural heart disease (4/49). Twelve percent of the children had a patent foramen ovale. None of the cardiac findings required therapeutic intervention other than repositioning of indwelling lines (6/49) due to intracardiac positioning. CONCLUSIONS: In our experience, findings on echocardiograms in childhood HR-ALL did not impact anthracycline administration. This study suggests that induction chemotherapy should not be delayed for an echocardiogram. However, whenever possible, a pre-therapy echocardiogram is still recommended for determining baseline function and to identify associated problems like pericardial effusions which were common in this study.

Mitral valve diseases in Williams syndrome-case report and review of the literature.

Williams syndrome is a genetic syndrome involving an unusual facies, short stature, developmental delay and heart defects. There is a genetic marker for this disease. Williams syndrome is frequently associated with congenital heart defects. The most common cardiac diagnoses are supravalve aortic stenosis, supravalve pulmonic stenosis, and arterial hypertension. In contrast, the association of mitral valve prolapse with Williams syndrome is less well defined. We present a case of a 15-year-old girl with Williams syndrome who underwent successful mitral valve repair. Review of the echocardiographic database of our institution over a 10-year period identified 26 other patients with Williams syndrome. Overall, 10 of the 27 children with Williams syndrome had mitral valve disease (37%) including 9 patients with mitral valve prolapse and one with mitral insufficiency. In conclusion, patients with Williams syndrome should be examined for mitral valve disease. Mitral valve repair is feasible and may be considered in the growing child with Williams syndrome.

Cardiac tumor in juvenile onset Behçet's disease: case report and review of the literature.

Behçet's disease is a chronic multisystemic inflammatory disorder characterized by orogenital ulcerations, uveitis, and occasional cardiac involvement. This report describes an atypical presentation of pediatric Behçet's disease with pulmonary emboli and a cardiac mass. A 16-year-old boy with a 2-year history of oral ulcers presented with weight loss, fever, joint pain, and a large tumor adhering to the free wall of the heart's right ventricle. Surgical biopsy demonstrated endomyocarditis with thrombus formation and led to the diagnosis. At the 2-year follow-up evaluation, intracardiac thrombus and pulmonic emboli had not recurred. Symptom control with immunosuppressive therapy was good.

Segmental differences of impaired diastolic relaxation following cardiopulmonary bypass surgery in children: a tissue Doppler study.

Impaired myocardial relaxation is an important aftereffect of cardiopulmonary bypass (CPB). Infants with their immature calcium metabolism may be particularly vulnerable. However, it has been difficult to quantitate diastolic dysfunction clinically. This study used tissue Doppler to measure regional diastolic myocardial velocities in 31 pediatric patients undergoing open heart surgery. Color tissue Doppler images were acquired in the operating room before and 8 and 24 h post CPB surgery. Early (E) and atrial (A) diastolic velocities were determined. Long axis motion was assessed from apical views near the mitral and tricuspid rings and radial wall motion from the parasternal view. The study included 31 children aged 3.6 +/- 4.4 years (6 days to 16 years), with a mean weight of 14.7 +/- 13.7 kg and body surface area of 0.59 +/- 0.35 m(2). Tissue Doppler analysis of regional wall motion revealed abnormal left ventricle (LV) and right ventricle (RV) diastolic relaxation in the early postoperative phase after CPB. Initially, all segments were significantly altered, but by 24 h, regional differences became apparent: LV radial wall motion was recovered, while longitudinal fibers in LV and RV appeared to be less resilient. RV myocardial mechanics were most abnormal. Tissue Doppler analysis may deepen our understanding of myocardial recovery and offers a sensitive tool to compare different cardioprotective strategies.

Coronary artery occlusion after arterial switch operation in an asymptomatic 15-year-old boy.

A 15-year-old boy with transposition of the great arteries (TGA) and neonatal arterial switch operation (ASO) presented with complete occlusion of the left main coronary artery (LMCA). Intra-operatively, an intramural left coronary artery was identified. Therefore, since age 7 years he had a series of screening exercise stress tests. At 13 years old, he had 3 to 4 mm ST segment depression in the infero-lateral leads without symptoms. This progressed to 4.2 mm inferior ST segment depression at 15 years old with normal stress echocardiogram. Sestamibi myocardial perfusion scan and cardiac magnetic resonance imaging was inconclusive. Therefore, a coronary angiogram was obtained which showed complete occlusion of the LMCA with ample collateralization from the right coronary artery system. This was later confirmed on a computed tomogram (CT) angiogram, obtained in preparation of coronary artery bypass grafting. The case illustrates the difficulty of detecting coronary artery stenosis and occlusion in young patients with rich collateralization. Coronary CT angiogram and conventional angiography were the best imaging modalities to detect coronary anomalies in this adolescent with surgically corrected TGA. Screening CT angiography may be warranted for TGA patients, particularly for those with known coronary anomalies.

Regional myocardial velocities and isovolumic contraction acceleration before and after device closure of atrial septal defects: a color tissue Doppler study.

BACKGROUND: The study analyzed the effect of atrial septal defect (ASD) device closure on regional wall motion in the right (RV) and left ventricles (LV) using color tissue Doppler imaging (TDI). Atrial septal defect closure results in acute volume unloading of the RV. For unknown reasons, some patients develop acute left-sided heart failure postintervention. METHODS: Color TDI was performed in 39 pediatric ASD and 75 age-matched controls. Regional wall motion in 5 LV and 1 RV segment were analyzed before, immediately after, and 24 hours after interventional ASD closure. Off-line postprocessing of echocardiographic data was used to determine myocardial velocities and acceleration during isovolumic contraction (IVA). Isovolumic contraction acceleration is the slope of the upstroke of the isovolumic contraction wave (IVA = peak velocity/acceleration time). RESULTS: At baseline, patients with ASD had significantly higher RV systolic velocities than controls. Isovolumic contraction acceleration was similar in patients with ASD and controls. In the catheterization laboratory postintervention, conventional function parameters remained stable but systolic myocardial velocities decreased significantly in all segments. Diastolic velocities fell in LV segments but not in the RV. In contrast to velocities, IVA was stable during ASD device closure. On follow-up at 24 hours, myocardial velocities had normalized. CONCLUSIONS: Device closure of ASD results to an acute transient decrease of regional myocardial velocities in the LV and RV, whereas the load-insensitive marker isovolumic acceleration remained stable. Therefore, the velocity changes may represent a response to altered left and right ventricular loading conditions. Color TDI is a sensitive tool to analyze ventricular mechanics.

Non-invasive detection of acute allograft rejection in children by tissue Doppler imaging: myocardial velocities and myocardial acceleration during isovolumic contraction.

BACKGROUND: In adults, an acute decrease of regional myocardial velocities is a sensitive marker of rejection. In children, velocities are more variable. A new marker, myocardial acceleration during isovolumic contraction (IVA), appears to be less age-dependent than myocardial velocities. This study therefore compared tissue Doppler (TDI)-derived velocities and IVA as potential rejection markers for children. METHODS: TDI was performed in 15 pediatric heart transplant recipients (age 8.0 +/- 3.6 years) during acute rejection and at baseline without rejection, 50 additional transplant children without rejection (7.8 +/- 5.9 years) and 30 age-matched healthy children (7.5 +/- 5.2 years). Color Doppler cine-loops of 3 cardiac cycles were stored as echocardiographic raw data. Using off-line post-processing, systolic (S) and diastolic (E) myocardial velocities and IVA were measured in 5 basal left ventricular segments. IVA is the peak isovolumic contraction wave velocity divided by acceleration time. RESULTS: Without rejection, transplant children had significantly lower diastolic velocities (basal lateral E 10.4 +/- 2.9 vs 11.9 +/- 2.6 cm/s; p < 0.001) and systolic velocities (S 5.6 +/- 1.4 vs 7.1 +/- 2.0 cm/s; p < 0.001) than normal age-matched controls, but IVA was similar (1.2 +/- 1.4 vs 1.3 +/- 0.5 m/s2). During rejection, all markers decreased significantly compared with age-matched normal control, the non-rejecting transplant group and individual baseline values. CONCLUSIONS: Regional myocardial velocities change significantly during acute allograft rejection in children. However, many children already have wall motion abnormalities at baseline, so results are often difficult to interpret. In contrast, isovolumic acceleration was normal without rejection and selectively decreased during the event. IVA is a promising non-invasive rejection marker for pediatric patients.

Double-orifice mitral valve with intact atrioventricular septum: an echocardiographic study with anatomic and functional considerations.

We identified 18 patients with double-orifice mitral valve (DOMV) and intact atrioventricular (AV) septum out of 40,179 echocardiographic studies performed between 1997 and 2002 at Children's Hospital, Denver, CO. In this study we describe (1) the anatomic characteristics of the DOMV in the absence of AV septal defect, (2) the function of the mitral valve by spectral and color Doppler flow mapping, and (3) associated lesions. The topographic location of the orifices in the leaflets suggests possible embryologic mechanisms of DOMV. In this series, DOMV was most commonly associated with left-sided obstructive lesions (in 39% of patients). Spectral and color Doppler interrogation demonstrated a normal flow profile in most cases; only 2 patients had significant mitral regurgitation or stenosis. Therefore, due to the uncertain natural history of this lesion and the potential need for endocarditis prophylaxis, careful imaging of the mitral valve is recommended, particularly in the presence of left-sided obstructive lesions.

The effect of pregestational diabetes on fetal heart function.

Pregestational diabetes affects nearly 2% of all pregnancies. Moreover, Type 2 diabetes in child-bearing women is on the rise because of the childhood obesity epidemic. Pregestational diabetes can affect the fetal heart in several ways. First, the risk of fetal congenital heart disease is markedly increased; second, fetal hypertrophic cardiomyopathy may occur even with good glycemic control; third, studies have shown impaired function of the hearts of some infants and fetuses of diabetic pregnancies, which can occur with and without septal hypertrophy. Small-for-gestational-age infants of diabetic mothers may have diminished cardiovascular health in the long term. This review mainly discusses methods to detect fetal diabetic cardiomyopathy prenatally. The focus is on the noninvasive diagnostic markers that can serve as an outcome measure for future therapeutic trials, which are still lacking. There is some experimental research on treatment strategies to prevent fetal heart disease in diabetic pregnancies but little clinical data.

Depression in adults with congenital heart disease-public health challenge in a rapidly expanding new patient population.

There is a growing population of adults with congenital heart disease (CHD) due to improved survival beyond childhood. It has been suggested that adults with CHD may be at increased risk for mental health problems, particularly depression. The reported incidence of depression in CHD varies from 9% to 30%. This review examines the evidence for a higher depression rate in CHD vs general population. Possible explanations are offered from a variety of disease models, ranging from brain injury to the psychoanalytical approach. Risk factors for an abnormal emotional adjustment and depression include early exposure to stress from illness and medical interventions in infancy, separation from the parents during hospitalizations and brain organic syndromes. Later in life, patients often have to cope with physical limitations. Recent improvements in care may be protective. Current patients may benefit from an earlier age at first surgical intervention, fewer reoperations and inclusion to the mainstream schooling, among other factors. At this point, there is little systematic knowledge about evidence-based therapeutic interventions for depression in adults with CHD. Health care providers of patients with CHD should be aware of mental health challenges and may take a more proactive approach to identifying patients at risk for depression.

Heart transplantation for congenital heart disease.

Congenital heart disease affects 0.8% of all live-born infants. Some of the malformed hearts can at best be palliated by conventional surgical or catheter interventions from the start. Others fail slowly from chronic overloading. Patients with congenital heart disease have been among the first transplant recipients since 1967. Primary therapy with infant heart transplant is a convincing concept from an immunological perspective but large-scale implementation is limited by donor organ shortages. Another growing area is rescue therapy for older patients with end-stage heart failure after palliative procedures, particularly those with single-ventricle hearts, systemic right ventricles, and associated arrhythmias.

Mechanical circulatory support for end-stage heart failure in repaired and palliated congenital heart disease.

Approximately one in one hundred children is born with congenital heart disease. Most can be managed with corrective or palliative surgery but a small group will develop severe heart failure, leaving cardiac transplantation as the ultimate treatment option. Unfortunately, due to the inadequate number of available donor organs, only a small number of patients can benefit from this therapy, and mortality remains high for pediatric patients awaiting heart transplantation, especially compared to adults. The purpose of this review is to describe the potential role of mechanical circulatory support in this context and to review current experience. For patients with congenital heart disease, ventricular assist devices are most commonly used as a bridge to cardiac transplantation, an application which has been shown to have several important advantages over medical therapy alone or support with extracorporeal membrane oxygenation, including improved survival to transplant, less exposure to blood products with less immune sensitization, and improved organ function. While these devices may improve wait list mortality, the chronic shortage of donor organs for children is likely to remain a problem into the foreseeable future. Therefore, there is great interest in the development of mechanical ventricular assist devices as potential destination therapy for congenital heart disease patients with end-stage heart failure. This review first discusses the experience with the currently available ventricular assist devices in children with congenital heart disease, and then follows to discuss what devices are under development and may reach the bedside soon.

The Ross operation in children and young adults: a fifteen-year, single-institution experience.

BACKGROUND: The optimal operation for aortic valve disease in children and young adults remains controversial. The Ross operation offers avoidance of anticoagulation and the potential for growth but is technically demanding and creates double-valve disease. The goal of this study is to report our experience with the Ross operation and the need for reintervention at intermediate follow-up. METHODS: A retrospective review of Ross operations in a single surgeon experience from 1992 to 2007 was conducted. All echocardiograms were reevaluated by a single cardiologist. RESULTS: The cohort included 54 patients with a mean age of 13.5 years (range 0.5 to 35 years). Pulmonary autograft implantation was accomplished using root replacement (n=43), root inclusion (n=9), and Dacron tube root replacement (n=2). Follow-up was available for 47 patients (87%) at a mean length of 6.4 years. There were no deaths. Kaplan-Meier estimates of freedom from explantation at 10 years were 100% for the autograft and 71% for the homograft. Autograft insufficiency at latest follow-up was trivial in 37 patients (82%), mild in 6 patients (13%), and moderate in 2 patients (4%). Reintervention for the homograft included balloon dilation in 3 children and conduit change in 5 children (all≤2 years old at initial operation). CONCLUSIONS: The Ross operation can be performed in children and adults with low mortality and can provide a durable result for the aortic valve with a low incidence of aortic insufficiency. The need for homograft replacement during follow-up in our series was primarily limited to children who were age 2 years or younger at initial operation.