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.

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.

In Vivo Hemodynamic Performance Evaluation of Novel Electrocardiogram-Synchronized Pulsatile and Nonpulsatile Extracorporeal Life Support Systems in an Adult Swine Model.

The primary objective of this study was to evaluate a novel electrocardiogram (ECG)-synchronized pulsatile extracorporeal life support (ECLS) system for adult partial mechanical circulatory support for adequate quality of pulsatility and enhanced hemodynamic energy generation in an in vivo animal model. The secondary aim was to assess end-organ protection during nonpulsatile versus synchronized pulsatile flow mode. Ten adult swine were randomly divided into a nonpulsatile group (NP, n = 5) and pulsatile group (P, n = 5), and placed on ECLS for 24 h using an i-cor system consisting of an i-cor diagonal pump, an iLA membrane ventilator, an 18 Fr femoral arterial cannula and a 23/25 Fr femoral venous cannula. Trials were conducted at a flow rate of 2.5 L/min using nonpulsatile or pulsatile mode (with assist ratio 1:1). Real-time pressure and flow data were recorded using a custom-based data acquisition system. To the best of our knowledge, the oxygenator and circuit pressure drops were the lowest for any available system in both groups. The ECG-synchronized i-cor ECLS system was able to trigger pulsatile flow in the porcine model. After 24-h ECLS, energy equivalent pressure, surplus hemodynamic energy, and total hemodynamic energy at preoxygenator and prearterial cannula sites were significantly higher in the P group than those in the NP group (P < 0.05). Urine output was higher in P versus NP (3379 ± 443 mL vs. NP, 2598 ± 1012 mL), and the P group seemed to require less inotropic support, but both did not reach statistical significances (P > 0.05). The novel i-cor system performed well in the nonpulsatile and ECG-synchronized pulsatile mode in an adult animal ECLS model. The iLA membrane oxygenator had an extremely lower transmembrane pressure gradient and excellent gas exchange capability. Our findings suggest that ECG-triggered pulsatile ECLS provides superior end-organ protection with improved renal function and systemic vascular tone.

Evaluation of a novel pulsatile extracorporeal life support system synchronized to the cardiac cycle: effect of rhythm changes on hemodynamic performance.

Arrhythmias are a frequent complication during extracorporeal life support (ECLS). A new ECLS system can provide pulsatile flow synchronized to the patient's intrinsic cardiac cycle based upon the R wave of the electrocardiogram (ECG). It is unclear how the occurrence of arrhythmias may alter the hemodynamic performance of the system. This in vitro study evaluated the effect of simulated arrhythmias on hemodynamics during R wave-triggered pulsatile ECLS. The ECLS circuit with an i-cor diagonal pump and iLA membrane ventilator was primed with whole blood at room temperature. Flow and pressure data were collected at 2.5 and 4 L/min for each condition using a customized data acquisition system. Pulsatile ECLS flow was R wave synchronized to an ECG simulator using 1:1, 1:2, and 1:3 assist ratios. Conditions tested included sinus rhythm at 45 and 90 bpm, supraventricular tachycardia (SVT), ventricular tachycardia (VT), and irregular rhythms such as ventricular fibrillation. Pulsatile mode was successfully triggered by ECG signals of normal sinus rhythm, SVT, VT, atrial fibrillation, atrial flutter, and ventricular bigeminy with assist ratios 1:1, 1:2, and 1:3. Regular rhythm at 90 bpm generated the best surplus hemodynamic energy (SHE). For SVT and VT, an assist ratio of 1:2 resulted in maximum pulsatile flow waveforms with optimal SHE at 2.5 L/min flow rate. At 4 L/min, SHE declined and the pressure drop increased independent of arrhythmia condition. Irregular rhythms still produced adequate pulsatile wave forms at lower pulsatile frequency. This study demonstrated the feasibility of generating pulsatile ECLS flow with the novel ECG-synchronized i-cor system during various simulated rhythms. The optimal rate for pulsatile flow was 90 bpm. During irregular rhythms, the lower pulsatile frequency was the more reliable synchronization mode for generating pulsatile flow.

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.

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.

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.

Tissue doppler myocardial velocity imaging in infants and children--a window into developmental changes of myocardial mechanics.

In adults, tissue Doppler myocardial velocity imaging (TDI) is a recommended component of routine echocardiography and particularly useful to assess diastolic function of the left ventricle. In contrast, color and pulsed-wave TDI velocities are less accepted in pediatrics, perhaps due to their strong age dependence in children. This review discusses the strengths and limitations of TDI velocity imaging in the pediatric age group. Myocardial velocities increase during normal childhood heart development, starting from fetal life, and these changes vary by cardiac segment. TDI velocity maturation opens an interesting window into the normal development of myocardial mechanics in childhood, but makes it difficult to interpret data in an individual child. Moreover, there is a wider range of normal for any given pediatric age than in adults. Still, TDI has been useful to monitor systolic heart function in children with cardiomyopathy or after heart transplantation. TDI studies revealed diastolic dysfunction in obese children and in cancer survivors with preclinical anthracycline cardiomyopathy. There is a growing body of studies using TDI to assess right heart function in children with congenital heart disease or pulmonary hypertension. Another potential strength of TDI velocities is the study of myocardial dyssynchrony where color TDI is well suited for rapid pediatric heart rates, even on fetal echocardiogram. Quantitative stress echocardiography with TDI is an emerging application in children that already offered insight into heart function in children with tetralogy of Fallot. Therefore, TDI velocity imaging should become part of the routine assessment of heart function in children.

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.

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.

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.

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.

Translational research in pediatric extracorporeal life support systems and cardiopulmonary bypass procedures: 2011 update.

Over the past 6 years at Penn State Hershey, we have established the pediatric cardiovascular research center with a multidisciplinary research team with the goal to improve the outcomes for children undergoing cardiac surgery with cardiopulmonary bypass (CPB) and extracorporeal life support (ECLS). Due to the variety of commercially available pediatric CPB and ECLS devices, both in vitro and in vivo translational research have been conducted to achieve the optimal choice for our patients. By now, every component being used in our clinical settings in Penn State Hershey has been selected based on the results of our translational research. The objective of this review is to summarize our translational research in Penn State Hershey Pediatric Cardiovascular Research Center and to share the latest results with all the interested centers.