The Effect of Cardiac Mass and Venous Return in Children with Postural Orthostatic Tachycardia Syndrome: A Prospective, Observational Study.

Low left ventricular mass index (LVMI) is thought to limit exercise tolerance in adult patients with postural orthostatic tachycardia syndrome (POTS). This finding has not been studied in children. We evaluated the effect of LVMI and hemodynamics at baseline and during exercise in POTS versus controls. POTS and control subjects aged 12-18 years were prospectively enrolled. POTS patients underwent autonomic studies. An echocardiogram was performed on all patients at baseline and during exercise. LVMI, venous return from inferior vena cava (IVC-VTI), left ventricular dimension, and cardiac output were assessed at baseline and during exercise. Generalized linear modeling with mixed effects was used to perform repeated measures testing between POTS and controls. Eighteen POTS patients (14 female, aged 15.4 ± 1.4 years) and nine control subjects (six female, aged 15.0 ± 1.3 years; p = 0.44) were enrolled. At baseline, LVMI was similar in both groups. During exercise, IVC-VTI, left ventricular end-diastolic dimension and volume, and stroke volume were lower in POTS patients. Peak heart rate was higher in POTS patients, but cardiac output was similar in both groups. Exercise time was higher in the control group (11.4 ± 2.7 min vs 9.2 ± 2.1, p = 0.024). Lower venous return resulted in smaller cardiac dimension and stroke volume during exercise. Higher heart rate in POTS may compensate to achieve similar cardiac output compared with control subjects. Lower ventricular filling and earlier time to peak heart rate may explain lower exercise capacity in pediatric POTS.

MRI detection of occult venous anomalies in a patient with Williams syndrome: a case report.

Williams syndrome is a multisystem, congenital disorder which is commonly associated with arterial stenoses: supravalvar aortic stenosis and peripheral pulmonary artery stenosis. Venous abnormalities have not been previously reported in children with Williams syndrome. We present a case of a 3-year-old girl with Williams syndrome and diffuse venous ectasia as detected by MRI.

Pediatric Electrocardiograms for the General Practitioner: The Importance of the T-Wave.

Electrocardiograms may be used to screen for cardiovascular disease (CVD) in children. Many forms of CVD that predispose to sudden cardiac death are associated with T-wave abnormalities in childhood. The T-wave is generated during ventricular repolarization. T-waves on an electrocardiogram typically undergo maturational changes throughout childhood and adolescence. A pediatric practitioner's ability to understand these changes is required to understand abnormal T-waves and recognize potentially life-threatening types of CVD. This review article uses a series of illustrative cases to highlight CVD associated with T-wave abnormalities in childhood. [Pediatr Ann. 2018;47(3):e106-e111.].

Risk Factors and Clinical Outcomes in Preterm Infants with Pulmonary Hypertension.

BACKGROUND: Pulmonary hypertension (PH) is a significant cause of morbidity in preterm infants, but no screening guidelines exist. We sought to identify risk factors and clinical outcomes associated with PH in preterm infants to develop a PH risk score. METHODS: Retrospective analysis of two separate populations of preterm infants (NICU cohort n = 230; Clinic registry n = 580). RESULTS: 8.3% of the NICU cohort had PH after 4 weeks of age, while 14.8% of the clinic registry had PH after 2 months of age. Lower birth weights and longer initial hospitalizations were associated with PH in both populations (p<0.001 for all tests). Using adjusted logistic regression, patent ductus arteriosus (PDA) requiring ligation was associated with PH in both the NICU cohort (OR: 3.19; p = 0.024) and the clinic registry (OR: 2.67; p<0.001). Risk factors (birth weight ≤780 grams, home supplemental oxygen use, and PDA ligation) identified in the clinic registry (training dataset) were validated in the NICU cohort with 0-1 factors present were associated with ≤1.5% probability of having PH, any 2 factors with a 25% probability, and all 3 factors with a 40% probability. CONCLUSIONS: Lower birth weight, PDA ligation, and respiratory support were associated with PH in both populations. A PH risk score based on clinical indicators from the training dataset predicted PH in the validation set. This risk score could help focus resources to preterm infants at higher risk for PH. Further work is needed to determine whether earlier or more aggressive management of ductal lesions could alter PH outcomes.

Pediatric pulmonary hypertension: diagnosis and management.

PURPOSE OF REVIEW: The article is intended to provide an overview of the most current modalities for the diagnosis and management of pulmonary hypertension in the pediatric population. RECENT FINDINGS: The WHO has recently updated the classification for pulmonary hypertension to include an expanded range of pediatric disease cohorts. We continue to gain knowledge of traditional pulmonary hypertension therapies (phosphodiesterase inhibitors, endothelin-receptor antagonists, and prostanoids) and remain optimistic that new therapeutic modalities [cyclic guanosine monophosphate (cGMP) stimulators, oral prostacyclins, and gene therapy] will lead to improved outcomes in pediatric patients. SUMMARY: Pediatric pulmonary hypertension is a heterogeneous disorder that has the potential to improve in some cases as children grow throughout childhood. Utilization of dedicated multidisciplinary teams of medical providers is necessary to deliver the highest level of medical care to this complex patient population. Ongoing development of enhanced screening protocols, novel disease-specific therapeutic targets, and comprehensive registries will hopefully lead to improved morbidity and mortality in the future.

Oxygen requirement as a screening tool for the detection of late pulmonary hypertension in extremely low birth weight infants.

BACKGROUND: Many extremely low birth weight infants develop pulmonary hypertension late in their clinical course, and over 60% go undetected by early screening echocardiography. At present, no standardised screening protocol exists for detecting late pulmonary hypertension in extremely low birth weight infants. We assessed the utility of oxygen supplementation as a predictor of late pulmonary hypertension. METHODS: A retrospective single-centre review of extremely low birth weight infants with no evidence of CHD and those surviving for >30 days was performed. The association between oxygen ⩾30% at day of life 30 and diagnosis of late pulmonary hypertension was estimated with an odds ratio and 95% confidence interval using logistic regression. Doppler echocardiography was used to diagnose pulmonary hypertension in the infants. RESULTS: A total of 230 infants met the study criteria. The incidence of late pulmonary hypertension was 8.3% (19/230). Infants with late pulmonary hypertension were more likely to have a lower mean birth weight (667.1±144 versus 799.3±140 g, p=0.001) and more likely to be small for gestational age (47.4 versus 14.2%, p=0.004). Oxygen requirement ⩾30% at day of life 30 was associated with increased risk of late pulmonary hypertension (odds ratio=3.77, 95% confidence interval=1.42-10.00, p=0.008) in univariate analysis and after adjusting for birth weight (odds ratio=2.47, 95% confidence interval=0.89-6.84, p=0.08). CONCLUSIONS: The need of oxygen supplementation ⩾30% at day of life 30 may be a good screening tool for detecting late pulmonary hypertension in extremely low birth weight infants.

Characterization of the pediatric acute-onset neuropsychiatric syndrome phenotype.

OBJECTIVE: Pediatric acute-onset neuropsychiatric syndrome (PANS) is a subtype of obsessive compulsive disorder (OCD) marked by an abrupt onset or exacerbation of neuropsychiatric symptoms. We aim to characterize the phenotypic presentation of youth with PANS. METHODS: Forty-three youth (ages 4-14 years) meeting criteria for PANS were assessed using self-report and clinician-administered measures, medical record reviews, comprehensive clinical evaluation, and laboratory measures. RESULTS: Youth with PANS presented with an early age of OCD onset (mean=7.84 years) and exhibited moderate to severe obsessive compulsive symptoms upon evaluation. All had comorbid anxiety and emotional lability, and scored well below normative means on all quality of life subscales. Youth with elevated streptococcal antibody titers trended toward having higher OCD severity, and presented more frequently with dilated pupils relative to youth without elevated titers. A cluster analysis of core PANS symptoms revealed three distinct symptom clusters that included core characteristic PANS symptoms, streptococcal-related symptoms, and cytokine-driven/physiological symptoms. Youth with PANS who had comorbid tics were more likely to exhibit a decline in school performance, visuomotor impairment, food restriction symptoms, and handwriting deterioration, and they reported lower quality of life relative to youth without tics. CONCLUSIONS: The sudden, acute onset of neuropsychiatric symptoms, high frequency of comorbidities (i.e., anxiety, behavioral regression, depression, and suicidality), and poor quality of life capture the PANS subgroup as suddenly and severely impaired youth. Identifying clinical characteristics of youth with PANS will allow clinicians to diagnose and treat this subtype of OCD with a more strategized and effective approach.

A high school-based voluntary cardiovascular risk screening program: issues of feasibility and correlates of electrocardiographic outcomes.

Risk factors for adult cardiovascular events can be identified from the prenatal period through childhood. We performed a cardiovascular risk-screening program in students from grades 9-12 in 7 high schools in Hillsborough County, FL. We obtained blood pressure (BP) measurements and calculated body mass index (BMI) as risk factors for future cardiovascular events as well as obtained an electrocardiogram (ECG) for the purposes of detecting possible life-threatening arrhythmias. Of ~14,000 students contacted, 600 (4 %) participated in the screening. Of these, 517 (86 %) were diagnosed with normal, 71 (12 %) with borderline, and 12 (1 %) with abnormal ECGs. Although no participant had any cardiac history, two of the abnormal ECGs indicated a cardiac diagnosis associated with the potential for sudden cardiac death. Both systolic and diastolic BP increased as the ECG diagnosis moved from normal (115.6/73.8) through borderline (121.0/75.9) to an abnormal (125.0/80.7) diagnosis (all P ≤ .0016). An increase in BMI was only observed when an ECG diagnosis was abnormal (P = .0180). Boys had a greater prevalence (18.97 %) of borderline or abnormal ECGs compared with girls (6.75 %), whereas no discernible differences were seen in ECG diagnosis between white and nonwhite individuals (15.09 and 12.26 %, respectively). Although participation rates were low, a high school-based cardiovascular risk-screening program including ECG is feasible. Although ECG diagnosis tended to be related to other known cardiovascular risk factors (BP, BMI), the utility of an abnormal ECG in adolescence as a predictor of future cardiovascular risk will require further evaluation in more controlled settings.

Unrepaired tetralogy of fallot with major aortopulmonary collateral arteries in an adult patient.

Tetralogy of Fallot is characterized by a ventricular septal defect, a large, overriding aorta, subpulmonic stenosis, and right ventricular hypertrophy. These lesions can be associated with abnormal development of the pulmonary vasculature. This can include peripheral pulmonic stenosis, discontinuous pulmonary arteries, anomalous pulmonary venous return, and the development of aortopulmonary collateral vessels. Aortopulmonary collateral vessels develop to supply underperfused areas of the pulmonary bed and pose a unique and challenging problem at the time of surgical repair, which involves closure of the ventricular septal defect, relief of right ventricular outflow tract obstruction, maintenance of pulmonary valve competency when possible, and establishment of laminar pulmonary blood flow to all segments of the pulmonary bed. We describe a 36-year-old man with unrepaired tetralogy of Fallot with distinctive aortopulmonary collaterals, who underwent complete surgical repair with good outcome. Two-dimensional echocardiogram, cardiac magnetic resonance imaging, and cardiac catheterization each provided vital details allowing a stepwise approach to defining his unique anatomy for surgical correction.

The importance of small for gestational age in the risk assessment of infants with critical congenital heart disease.

BACKGROUND: Infants with critical congenital heart disease who require cardiothoracic surgical intervention may have significant post-operative mortality and morbidity. Infants who are small for gestational age <10th percentile with foetal growth restriction may have end-organ dysfunction that may predispose them to increased morbidity or mortality. METHODS: A single-institution retrospective review was performed in 230 infants with congenital heart disease who had cardiothoracic surgical intervention <60 days of age. Pre-, peri-, and post-operative morbidity and mortality markers were collected along with demographics and anthropometric measurements. RESULTS: There were 230 infants, 57 (23.3%) small for gestational age and 173 (70.6%) appropriate for gestational age. No significant difference was noted in pre-operative markers - gestational age, age at surgery, corrected gestational age, Society for Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality score; or post-operative factors - length of stay, ventilation days, arrhythmias, need for extracorporeal membrane oxygenation, vocal cord dysfunction, hearing loss; or end-organ dysfunction - gastro-intestinal, renal, central nervous system, or genetic. Small for gestational age infants were more likely to have failed vision tests (p = 0.006). Small for gestational age infants were more likely to have increased 30-day (p = 0.005) and discharge mortality (p = 0.035). Small for gestational age infants with normal birth weight (>2500 g) were also at increased risk of 30-day mortality compared with appropriate for gestational age infants (p = 0.045). CONCLUSIONS: Small for gestational age infants with congenital heart disease who undergo cardiothoracic surgery <60 days of age have increased risk of mortality and failed vision screening. Assessment of foetal growth restriction as part of routine pre-operative screening may be beneficial.

Surgical repair of atrial septal defect with severe pulmonary hypertension during pregnancy: a case report with literature review.

We are reporting a case of a 37-year-old pregnant woman with a large secundum atrial septal defect with left-to-right shunt and severe pulmonary hypertension. Her atrial septal defect was undiagnosed before this pregnancy. After carefully considering all the options, we repaired her atrial septal defect with an open heart surgical closure at 20 weeks of gestation. A substantial and consistent reduction in pulmonary arterial pressure after the surgery and subsequent uneventful delivery indicate that surgical repair of atrial septal defects is a viable option that should be considered for such patients.

Long-term management of adults with conotruncal lesions: the diagnostic approach at All Children's Hospital.

Improved survival in children with complex congenital cardiac disease, such as conotruncal abnormalities, has created a sub-population of children and young adults who need comprehensive multi-disciplinary long-term follow-up. Routine surveillance with comprehensive screening for structural heart disease, functional heart disease, thromboembolic disease, arrhythmias, and associated end-organ dysfunction is important. Future research will better define the care plans for routine surveillance in patients with conotruncal abnormalities.

Long-term management of patients with hypoplastic left heart syndrome: the diagnostic approach at All Children's Hospital.

Improved survival in children with hypoplastic left heart syndrome has created a sub-population of children and young adults who are living with functionally univentricular physiology. Routine surveillance with comprehensive screening for structural cardiac disease, functional cardiac disease, arrhythmias, thromboembolic disease, and associated dysfunction of end organs is important. Future directives will better define the plans of care for routine surveillance in patients with hypoplastic left heart syndrome.

Lessons learned from 119 consecutive cardiac transplants for pediatric and congenital heart disease.

BACKGROUND: This manuscript reviews all patients who underwent orthotopic heart transplantations (OHT) at our program (116 patients underwent 119 OHT) to describe their diagnostic characteristics and to assess risk factors for mortality. METHODS: Median age at OHT was 179 days (mean, 1,446.6 ± 188.9 days [4.0 ± 0.5 years]; range, 5 days to 7,125 days [19.5 years]; 15 neonates, 68 infants). Median weight at OHT was 5.5 kg (mean, 17.2 ± 2.1 kg; range, 2.2 to 113 kg). Diagnoses were cardiomyopathy (n = 37), primary transplantation for hypoplastic left heart syndrome (HLHS) or HLHS-related malformation (n = 29), transplantation after prior cardiac surgery for HLHS or HLHS-related malformation (n = 9), non-HLHS congenital heart disease (n = 39), and retransplant (n = 5). RESULTS: Overall Kaplan-Meier 5-year survival was 72.7%. Operative mortality was 12.6% (15 patients). Late mortality was 13.4% (16 patients). Eighty-five patients survived, with a mean follow-up of 5.76 ± 0.48 years (median, 5.1 years; range, 0.12 to 14.0 years). Total follow-up was 507.0 years. No survival difference was seen among the five diagnostic subgroups (p = 0.20). Univariate association between risk factors and survival was assessed for the following variables: age (p = 0.91), weight (p = 0.86), sex (p = 0.47), race (p = 0.40), insurance classification (p = 0.42), high PRA (p = 0.20), pretransplant mechanical circulatory support (p < 0.001), posttransplant mechanical circulatory support (p < 0.001), redo sternotomy (p = 0.07), heterotaxy (p = 0.02), cardiopulmonary bypass time (p = 0.01), and donor heart cross-clamp time (p = 0.02). CONCLUSIONS: Excellent results are expected for children undergoing OHT regardless of diagnostic classification. Pretransplant mechanical circulatory support, posttransplant mechanical circulatory support, cardiopulmonary bypass time, donor heart cross-clamp time, and heterotaxy are risk factors for decreased survival.

Blalock-taussig shunt thrombosis prophylaxis in a patient with jacobsen syndrome and thrombocytopenia.

Jacobsen syndrome (JS) is a rare chromosomal anomaly caused by deletions in the distal long arm of chromosome 11. Features of the syndrome include growth and developmental delays, a distinctive facial appearance, and a variety of physical problems including heart defects and bleeding disorders. Congenital heart defects occur in approximately 50% of children with JS. Hypoplastic left heart syndrome (HLHS) has been occasionally reported in association with JS. In such cases, the hematological abnormalities may influence the outcome from single-ventricle palliation through staged surgical reconstruction. Thrombotic obstruction or occlusion of the modified Blalock-Taussig (BT) shunt is a well-documented cause of interstage mortality following the Norwood operation. Although there is no consensus regarding the therapeutic value of antiplatelet therapy during the interstage period following the first stage of palliation, maintenance of shunt patency is critically important. For patients with JS undergoing single-ventricle palliation, decisions regarding antiplatelet therapy during the interstage period may be further complicated by the presence of thrombocytopenia and platelet dysfunction related to JS. We report the case of a patient with HLHS, JS, and thrombocytopenia who underwent the Norwood procedure, and we describe our strategy for prophylaxis against thrombosis of the BT shunt.

Mesalamine-induced myocarditis and coronary vasculitis in a pediatric ulcerative colitis patient: a case report.

Mesalamine-containing products are often a first-line treatment for ulcerative colitis. Severe adverse reactions to these products, including cardiovascular toxicity, are rarely seen in pediatric patients. We present a case of a 16-year-old boy with ulcerative colitis treated with Asacol, a mesalamine-containing product, who developed sudden onset chest pain after four weeks on therapy. Serial electrocardiograms showed nonspecific ST segment changes, an echocardiogram showed mildly decreased left ventricular systolic function with mild to moderate left ventricular dilation and coronary ectasia, and his troponins were elevated. Following Asacol discontinuation, his chest pain resolved, troponins were trending towards normal, left ventricular systolic function normalized, and coronary ectasia improved within 24 hours suggesting an Asacol-associated severe drug reaction. Mesalamine-induced cardiovascular toxicity, although rare, may represent a life-threatening disorder. Therefore, every patient presenting with acute chest pain should receive a workup to rule out this rare drug-induced disorder.

Intensive care and perioperative management of patients with complete atrioventricular septal defect.

Operative repair of an atrioventricular septal defect is not without risk. The purpose of this review is to highlight various key topics in the perioperative treatment of patients with atrioventricular septal defects, including challenges related to Down syndrome, postoperative arrhythmias, pulmonary hypertension, hypothyroidism, postoperative residual and recurrent lesions, including systemic atrioventricular valvar regurgitation and left ventricular outflow tract obstruction, sedation and analgesia, and vascular access.

Magnetic resonance angiography detection of vascular aneurysms in patients with kawasaki disease and coronary artery aneurysms.

Kawasaki disease is a systemic panvasculitis that causes coronary artery aneurysms in approximately 15% to 25% of untreated patients. Systemic vascular aneurysms may also occur in medium-sized arteries throughout the body and may lead to increased morbidity and mortality in patients with Kawasaki disease. We report a case of diffuse systemic aneurysm formation in a 2-year-old patient with Kawasaki disease with coronary artery aneurysms. Full-body magnetic resonance angiography (MRA) imaging was utilized both in the acute phase and again prior to cardiac catheterization performed at 6 months from the acute illness. The initial MRA detected aneurysmal dilatation of the common and internal iliac arteries bilaterally in the acute phase. Subsequent MRA performed prior to cardiac catheterization 6 months later demonstrated resolution of the iliac artery lesions. Full-body MRA may be useful in screening for associated systemic vascular aneurysms in patients with Kawasaki disease and associated coronary artery aneurysms.

Rescue cardiac transplantation for failing staged palliation in patients with hypoplastic left heart syndrome.

OBJECTIVE: Orthotopic heart transplantation is considered a rescue option for children with failing staged palliation or repair of hypoplastic left heart syndrome. We present our strategy for management, and outcomes, for these complex patients. METHODS: We transplanted 68 consecutive children, with diagnoses of hypoplastic left heart syndrome in 31, cardiomyopathy in 20, and post-operative complex congenital heart disease in 17. Of these patients, 9 (13.2%) were neonates, and 46 (67.6%) were infants. Median age was 118.5 days. Operative technique involves bicaval cannulation and anastamoses with continuous low flow bypass, and either short periods of circulatory arrest or continuous low flow antegrade cerebral perfusion for reconstruction of the aortic arch. Initial reperfusion of the donor heart utilizes glutamate and aspartate substrate enriched white blood cell filtered cardioplegia. Immunosuppressive therapy includes induction (pulse steroids, gamma globulin, and polyclonal rabbit antithymocyte globulin) and initial maintenance (calcineurin inhibitor, an anti-proliferative agent, and a weaning steroid protocol). Of the 31 patients with hypoplastic left heart syndrome, 23 underwent primary transplantation, and 8 underwent rescue transplantation from failing staged palliation in seven, or attempted biventricular repair in one. Of the seven patients who had failing staged palliation, three had undergone only the Norwood Stage 1 operation, 2 had undergone a Norwood Stage 1 operation and a Glenn superior cavopulmonary anastomosis and two had undergone a Norwood Stage 1 operation, a Glenn superior cavopulmonary anastomosis, and a completion Fontan operation. RESULTS: The group undergoing primary transplantation was younger (p equals 0.007), weighed less (p equals 0.003), and waited longer for an appropriate donor heart (p equals 0.021) compared to those requiring rescue transplantation. No significant difference exists between the groups with regards to donor heart ischaemic time or post-transplant length of hospital stay. Thirty day survival (p equals 0.156) and overall survival (p equals 0.053) was better in those having primary transplantation, although these differences were not statistically significant when a p value of less than 0.05 is considered to be significant. In those having primary transplantation, no patients had elevated panel reactive antibody greater than 10%. Half of the 8 requiring rescue transplantation had panel reactive antibody greater than 10%, and this subgroup did especially poorly. CONCLUSION: Cardiac transplantation can offer children with failing staged palliation their only chance of survival. Transplantation, however, carries a high risk in this subgroup, especially in the setting of elevated panel reactive antibody.