StethAid: A Digital Auscultation Platform for Pediatrics.

(1) Background: Mastery of auscultation can be challenging for many healthcare providers. Artificial intelligence (AI)-powered digital support is emerging as an aid to assist with the interpretation of auscultated sounds. A few AI-augmented digital stethoscopes exist but none are dedicated to pediatrics. Our goal was to develop a digital auscultation platform for pediatric medicine. (2) Methods: We developed StethAid-a digital platform for artificial intelligence-assisted auscultation and telehealth in pediatrics-that consists of a wireless digital stethoscope, mobile applications, customized patient-provider portals, and deep learning algorithms. To validate the StethAid platform, we characterized our stethoscope and used the platform in two clinical applications: (1) Still's murmur identification and (2) wheeze detection. The platform has been deployed in four children's medical centers to build the first and largest pediatric cardiopulmonary datasets, to our knowledge. We have trained and tested deep-learning models using these datasets. (3) Results: The frequency response of the StethAid stethoscope was comparable to those of the commercially available Eko Core, Thinklabs One, and Littman 3200 stethoscopes. The labels provided by our expert physician offline were in concordance with the labels of providers at the bedside using their acoustic stethoscopes for 79.3% of lungs cases and 98.3% of heart cases. Our deep learning algorithms achieved high sensitivity and specificity for both Still's murmur identification (sensitivity of 91.9% and specificity of 92.6%) and wheeze detection (sensitivity of 83.7% and specificity of 84.4%). (4) Conclusions: Our team has created a technically and clinically validated pediatric digital AI-enabled auscultation platform. Use of our platform could improve efficacy and efficiency of clinical care for pediatric patients, reduce parental anxiety, and result in cost savings.

Double Aortic Arch with Atresia of the Left Aortic Arch Proximal to the Left Common Carotid Artery: First Report of Documentation by Surgical Observation.

Double aortic arch associated with atresia of the left arch proximal to the left common carotid artery has been considered a theoretical possibility. To our knowledge, we report the first patient with this anatomy confirmed by surgical observation.

Coarctation of the Aorta: Delay in Diagnosis and Referral Basis from Infancy to Adulthood.

OBJECTIVES: To assess the role of neonatal pulse oximetry screening and other bases of referral for patients of all ages with aortic coarctation referred to a tertiary care medical center. STUDY DESIGN: Medical records were reviewed for 200 consecutive patients diagnosed and treated for CoA in either 2006-2011 or 2015-2019, before and after mandated pulse oximetry screening, respectively. RESULTS: In both groups, ∼50% of patients were diagnosed within the first 5 days. Diagnosis by fetal echocardiography was more frequent in the 2015-2019 group (30.5% vs 20.5%; P < .03); obstruction often developed only as the ductus arteriosus closed. In each group, ∼25% of patients were diagnosed at age >1 year and 7% had an incidental diagnosis. Pulse oximetry screening was documented as abnormal in only 8 of 47 patients and was not performed in those with a fetal diagnosis. Evaluation of a murmur was the second most frequent basis for referral. Moderate to severe left ventricular dysfunction occurred mainly in infants in the first month, with a similar frequency in the 2 groups; these patients often had tachypnea or poor weight gain. Decreased femoral pulses or systemic hypertension were infrequently documented by referring physicians. Hypertension typically was ascribed to a renal or essential basis. Exercise symptoms occurred mainly in patients age >10 years. CONCLUSIONS: Although fetal echocardiography and neonatal pulse oximetry contribute to the diagnosis of coarctation, physical examination has an important complementary role. Evaluation of peripheral pulses on initial and early follow-up neonatal examinations, along with consideration of coarctation in any patient with hypertension, are needed to improve timely detection.

Scimitar syndrome: A new multipatch technique and incidence of postoperative pulmonary vein obstruction.

OBJECTIVE: A review of our center's experience before March 2011 showed that one half of 36 patients who had a baffling or reimplantation procedure to repair scimitar syndrome developed pulmonary vein obstruction. We analyzed the results of a new operation that enlarges the left atrium and avoids circuitous pathways or tension on the scimitar pulmonary vein. METHODS: Between April 2011 and November 2018, 22 patients underwent scimitar vein surgery; 11 had baffling or reimplantation and 11 only had the new operation that included resection of the atrial septum with removal of the muscular limbus. The left atrium was pulled down toward the scimitar vein and a V-shaped incision made at the scimitar vein atrial junction with the space filled with a pulmonary homograft. If the scimitar vein coursed adjacent to the atrium, a V-shaped incision was made into the scimitar vein and directly anastomosed to the atrium. A patch of autologous pericardium was used to septate the atrium and an additional patch placed anteriorly to augment the inferior vena cava. RESULTS: Of the 11 patients who had baffling or reimplantation, 5 developed pulmonary vein obstruction between 45 days and 9.5 months after surgery associated with baffle thrombosis or tension on the pulmonary vein. None of the 11 patients who only had the new procedure developed pulmonary vein obstruction during postoperative monitoring up to 3.6 years. CONCLUSIONS: Patients having only the multipatch procedure for repair of scimitar syndrome have not developed postoperative pulmonary vein obstruction in the short to intermediate term.

Clinical Detection of Hemodynamically Significant Isolated Secundum Atrial Septal Defect.

The diagnosis of secundum atrial septal defect often is delayed. For 310 patients with hemodynamically significant secundum atrial septal defect undergoing closure over a 5-year period at a single medical center, this study reviews the symptoms prompting referral, limitations of physical examination and electrocardiography, and basis for initially missing the diagnosis.

Needles in Hay II: Detecting Cardiac Pathology by the Pediatric Chest Pain Standardized Clinical Assessment and Management Plan.

OBJECTIVES: To determine if patients evaluated using the pediatric chest pain standardized clinical assessment and management plan (SCAMP) in cardiology clinic were later diagnosed with unrecognized cardiac pathology, and to determine if other patients with cardiac pathology not enrolled in the SCAMP would have been identified using the algorithm. STUDY DESIGN: Patients 7-21 years of age, newly diagnosed with hypertrophic or dilated cardiomyopathy, coronary anomalies, pulmonary embolus, pulmonary hypertension, pericarditis, or myocarditis were identified from the Boston Children's Hospital (BCH) cardiac database between July 1, 2010 and December 31, 2012. Patients were cross-referenced to the SCAMP database or retrospectively assessed with the SCAMP algorithm. RESULTS: Among 98 patients with cardiac pathology, 34 (35%) reported chest pain, of whom 10 were diagnosed as outpatients. None of these patients were enrolled in the SCAMP because of alternate chief complaints (n = 4) or referral to BCH for management of the new diagnosis (n = 6). Each of these patients would have had an echocardiogram recommended by retrospective application of the SCAMP algorithm. Two other patients with cardiac pathology were among the 1124 patients assessed by the SCAMP. One patient initially diagnosed with noncardiac chest pain presented 18 months later and was diagnosed with myocarditis as an inpatient. One patient seen initially in the emergency department was subsequently diagnosed with pericarditis as an outpatient. CONCLUSIONS: Patients assessed by the chest pain SCAMP at BCH were not later diagnosed with cardiac pathology that was missed at the initial encounter. Nonenrolled outpatients with cardiac pathology and chest pain would have been successfully identified with the SCAMP algorithm.

Prenatal and newborn screening for critical congenital heart disease: findings from a nursery.

BACKGROUND: Delayed diagnosis of critical congenital heart disease (CCHD) in neonates increases morbidity and mortality. The use of pulse oximetry screening is recommended to increase detection of these conditions. The contribution of pulse oximetry in a tertiary-care birthing center may be different from at other sites. METHODS: We analyzed CCHD pulse oximetry screening for newborns ≥ 35 weeks' gestation born at Brigham and Women's Hospital and cared for in the well-infant nursery during 2013. We identified patients with prenatal diagnosis of CCHD. We also identified infants born at other medical centers who were transferred to Boston Children's Hospital for CCHD and determined if the condition was diagnosed prenatally. RESULTS: Of 6838 infants with complete pulse oximetry data, 6803 (99.5%) passed the first screening. One infant failed all 3 screenings and had the only echocardiogram prompted by screening that showed persistent pulmonary hypertension. There was 1 false-negative screening in an infant diagnosed with interrupted aortic arch. Of 112 infants born at Brigham and Women's Hospital with CCHD, 111 had a prenatal diagnosis, and none was initially diagnosed by pulse oximetry. Of 81 infants transferred to Boston Children's Hospital from other medical centers with CCHD, 35% were diagnosed prenatally. CONCLUSIONS: In our tertiary-care setting, pulse oximetry did not detect an infant with CCHD because of effective prenatal echocardiography screening. Pulse oximetry will detect more infants in settings with a lower prenatal diagnosis rate. Improving training in complete fetal echocardiography scans should also improve timely diagnosis of CCHD.

Regional implementation of a pediatric cardiology chest pain guideline using SCAMPs methodology.

BACKGROUND AND OBJECTIVES: Chest pain is a complaint for which children are frequently evaluated. Cardiac causes are rarely found despite expenditure of considerable time and resources. We describe validation throughout New England of a clinical guideline for cost-effective evaluation of pediatric patients first seen by a cardiologist for chest pain using a unique methodology termed the Standardized Clinical Assessment and Management Plans (SCAMPs). METHODS: A total of 1016 ambulatory patients, ages 7 to 21 years initially seen for chest pain at Boston Children's Hospital (BCH) or the New England Congenital Cardiology Association (NECCA) practices, were evaluated by using a SCAMPs chest pain guideline. Findings were analyzed for diagnostic elements, patterns of care, and compliance with the guideline. Results from the NECCA practices were compared with those of Boston Children's Hospital, a regional core academic center. RESULTS: Two patients had chest pain due to a cardiac etiology, 1 with pericarditis and 1 with an anomalous coronary artery origin. Testing performed outside of guideline recommendations demonstrated only incidental findings. Patients returning for persistent symptoms did not have cardiac disease. The pattern of care for the NECCA practices and BCH differed minimally. CONCLUSIONS: By using SCAMPs methodology, we have demonstrated that chest pain in children is rarely caused by heart disease and can be evaluated in the ambulatory setting efficiently and effectively using minimal resources. The methodology can be implemented regionally across a wide range of clinical practice settings and its approach can overcome a number of barriers that often limit clinical practice guideline implementation.

Outcome predictors and implications for management of scimitar syndrome.

BACKGROUND: Scimitar syndrome is a rare congenital anomaly. We evaluated risk factors for postoperative pulmonary vein stenosis or death and predictive factors for survival without scimitar vein surgery in patients with scimitar syndrome. METHODS: The records of patients with scimitar syndrome evaluated at our medical center between 1964 and 2011 were reviewed. RESULTS: Scimitar syndrome was identified in 80 patients, with a median follow-up of 4.5 years. Patients presenting less than 1 year of age had a higher incidence of symptoms, aortopulmonary collaterals, coexisting congenital heart disease (CHD), extracardiac anomalies, and pulmonary hypertension. Of 36 patients having scimitar vein surgery, 18 had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures, early or late in the study period, and tended to be more common in infants (P = .10). Overall, 19 (24%) of 80 died. Multivariate risk factors for death included systolic pulmonary pressure >0.5 systemic level (P = .007) and left pulmonary vein stenosis (P = .009). Pulmonary artery systolic pressure <0.5 systemic level (P = .01) and absence of CHD excluding atrial septal defect (P = .01) were predictive factors in 28 patients who survived and did not have scimitar vein surgery; these patients had no or mild right ventricular dilation and a ratio of pulmonary-to-systemic flow <1.6 either at baseline, after coiling aortopulmonary collaterals or nonscimitar vein intervention. CONCLUSIONS: Postoperative pulmonary vein obstruction is common after scimitar vein surgery regardless of redirection technique. Pulmonary hypertension and left pulmonary vein stenosis are risk factors for death, whereas patients without significant pulmonary hypertension or associated CHD did well without scimitar vein surgery. These observations may guide management decisions in patients with scimitar syndrome.

Outcomes of transcatheter approach for initial treatment of pulmonary atresia with intact ventricular septum.

OBJECTIVE: To review the short and medium term outcome of transcatheter pulmonary valve perforation (PVP) in patients with pulmonary atresia-intact ventricular septum and non-right ventricular dependant coronary circulation (PA/IVS non-RVDCC). BACKGROUND: PVP in patients with PA/IVS non-RVDCC has become more common in the past two decades. However, data on outcomes with this strategy are mixed. METHODS: Data were reviewed retrospectively for all patients with PA/IVS non-RVDCC treated from 1996- 2010 at our institution. Patients who had severe neonatal Ebstein malformation, or initial interventional management at another institution were excluded. RESULTS: PVP was attempted in 30 of 50 patients (60%); 26 (87%) of these had a successful procedure. Twenty-four patients (48%) had surgery without PVP. There were no deaths in the cohort. Complications of PVP included 5 (17%) myocardial perforations. Of those with successful PVP, 10 (38%) did not have surgery (PVP-NS) and 16 (62%) had surgery (PVP-S) prior to discharge. Tricuspid valve (TV) Z-score was larger in the PVP-NS than in PVP-S patients, with median TV diameter Z-scores of +0.7 (-0.9, 1.7) and -1.1 (-2.8, 2), respectively (P = 0.01). Time from PVP to either hospital discharge (PVP-NS group) or surgery (PVP-S group) was significantly different between groups: 15 (7, 22) and 8 days (0, 46), respectively (P = 0.01). There were no differences in the number of trials or lowest arterial PaO2 off prostaglandins between groups. All patients in the PVP-NS group had a biventricular circulation at a median follow-up of 4.3 years. CONCLUSIONS: The results of a collaborative approach to treating neonates with PA/IVS non-RVDCC are excellent. Smaller TV size is associated with greater likelihood of surgery prior to discharge, and may serve as a surrogate for early RV inadequacy.

Resource Utilization Reduction for Evaluation of Chest Pain in Pediatrics Using a Novel Standardized Clinical Assessment and Management Plan (SCAMP).

BACKGROUND: Chest pain is a common reason for referral to pediatric cardiologists. Although pediatric chest pain is rarely attributable to serious cardiac pathology, extensive and costly evaluation is often performed. We have implemented a standardized approach to pediatric chest pain in our pediatric cardiology clinics as part of a broader quality improvement initiative termed Standardized Clinical Assessment and Management Plans (SCAMPs). In this study, we evaluate the impact of a SCAMP for chest pain on practice variation and resource utilization. METHODS AND RESULTS: We compared demographic variables, clinical characteristics, and cardiac testing in a historical cohort (n=406) of patients presenting to our outpatient division for initial evaluation of chest pain in the most recent pre-SCAMP calendar year (2009) to patients enrolled in the chest pain SCAMP (n=364). Demographic variables including age at presentation, sex, and clinical characteristics were similar between groups. Adherence to the SCAMP algorithm for echocardiography was 84%. Practice variation decreased significantly after implementation of the SCAMP (P<0.001). The number of exercise stress tests obtained was significantly lower in the SCAMP-enrolled patients compared with the historic cohort (∼3% of patients versus 29%, respectively; P<0.001). Similarly, there was a 66% decrease in utilization of Holter monitors and 75% decrease in the use of long-term event monitors after implementation of the chest pain SCAMP (P=0.003 and P<0.001, respectively). The number of echocardiograms obtained was similar between groups. CONCLUSIONS: Implementation of a SCAMP for evaluation of pediatric chest pain has lead to a decrease in practice variation and resource utilization. (J Am Heart Assoc. 2012;1:jah3-e000349 doi: 10.1161/JAHA.111.000349.).

Needles in hay: chest pain as the presenting symptom in children with serious underlying cardiac pathology.

INTRODUCTION: Chest pain in children is common, but rarely heralds serious underlying cardiac pathology. Despite this, the anxiety of missing a potentially life threatening condition creates a large burden of referrals and diagnostic testing. We evaluated patients diagnosed with 1 of 9 serious cardiac diseases and detailed the clinical signs and symptoms of the patients presenting with chest pain. METHODS: Patients diagnosed between the ages of 7 and 21 years from January 2000 to December 2009 at Children's Hospital Boston (CHB) were identified from a database using diagnostic and billing codes for aortic dissection, coronary anomalies, dilated cardiomyopathy, hypertrophic cardiomyopathy, myocarditis, pericarditis, pulmonary embolus, pulmonary hypertension, and Takayasu arteritis. Patients with previously diagnosed congenital or acquired heart disease were excluded. RESULTS: Four hundred eighty-four patients were included and 35% presented with chest pain. Forty-one (24%) of these patients with chest pain were diagnosed in the outpatient cardiology clinic, while the remaining 130 patients (76%) were diagnosed in the emergency department (ED) or inpatient setting. Coronary artery anomalies were the most common diagnosis made in cardiology clinic, and 16 of the 23 (70%) patients with serious coronary anomalies had exercise-induced chest pain. Patients presenting to the ED or inpatient units tended to have other important nonspecific symptoms (35-44%), high-risk past medical histories (12%), physical examination findings (32%), and electrocardiogram (ECG) abnormalities (78%) that heighten clinical suspicion of cardiac disease. CONCLUSIONS: Identifying underlying cardiac pathology in the CHB outpatient cardiology department in patients presenting with chest pain is rare, with only 41 cases over a 10-year period. The presence of exertional chest pain was important in identifying patients with coronary artery anomalies. A detailed history and physical examination, along with a critical review of an ECG, seem to identify those patients with rare diseases who need further diagnostic testing.

Left ventricular diastolic heart failure in teenagers who underwent balloon aortic valvuloplasty in early infancy.

We report 4 teenage patients who, after successful palliation of severe aortic valve stenosis by balloon aortic valvuloplasty in early infancy, presented within the previous 3 years with significant left ventricular (LV) diastolic heart failure. All patients had remained asymptomatic until their teenage years and had normal or hyperdynamic LV systolic function on presentation, with limited residual aortic valve stenosis and regurgitation. All underwent echocardiography, cardiac catheterization, and cardiac magnetic resonance evaluation. One notable common feature in the 4 patients was the presence of a confluent layer of LV subendocardial hyperenhancement demonstrated by late gadolinium enhancement technique. Histopathology was available for 2 patients, which documented significant LV endocardial fibroelastosis. One patient who underwent endocardial fibroelastosis resection in conjunction with aortic valve replacement had late clinical improvement. In conclusion, this group of patients may represent an important emerging clinical entity and merits close clinical surveillance, with prospective assessment of diastolic function.

Factors associated with development of atrial septal restriction in patients with tricuspid atresia involving the right-sided atrioventricular valve.

BACKGROUND: Clinical practice is discrepant regarding routine enlargement of the interatrial communication (IAC) in patients with right-sided atrioventricular valve atresia. We determined the percentage and risk factors of those who develop a restrictive IAC. METHODS: Medical records were reviewed for patients treated from 1985 to 2006, including those admitted in the first 6 weeks of life (group A), and those referred at a later age (group B), some of whom had routine atrial septal procedures. In group A, we analyzed the initial postnatal echocardiogram. RESULTS: Group A consisted of 79 patients, 16 of whom had an atrial septectomy as part of the initial surgical procedure. Of the remaining 63 patients, 9 (14%) developed atrial septal restriction. In group A, an atrial septal aneurysm (ASA) (OR 16, P = .006) and IAC diameter < 5 mm (OR 13, P = .009) were associated with atrial septal restriction. Atrial septal restriction occurred in 80% of patients with both features, 20% with IAC > or = 5 mm and ASA, 18% with IAC < 5 mm and no ASA, and 2% with neither feature. Group B consisted of 95 patients, 27 of whom had an atrial septal procedure. Of the remaining 68 patients, 11 (16%) developed atrial septal restriction. CONCLUSION: Routine enlargement of the IAC is not necessary in patients with right-sided atrioventricular valve atresia. Patients with ASA or IAC < 5 mm are at increased risk for development of atrial septal restriction.

Endovascular treatment for superior vena cava occlusion or obstruction in a pediatric and young adult population: a 22-year experience.

OBJECTIVES: The purpose of this research was to investigate the causes and symptoms of superior vena cava (SVC) obstruction or occlusion and report on the long-term results of transcatheter therapy. BACKGROUND: Information on transcatheter therapy for SVC obstruction is limited. METHODS: Superior vena cava catheterization interventions between August 1984 and April 2006 were reviewed. Patients were divided into 2 subgroups depending on whether or not they had previously undergone congenital cardiac surgery. RESULTS: Sixty-three patients with median age of 3.7 years (range 1 month to 42 years) and weight of 13.3 kg (range 3 to 114 kg) were treated. Fifty patients (79%) were symptomatic, although only 50% had symptoms suggestive of SVC obstruction. Superior vena cava syndrome was more common in the non-cardiac surgical group (52% vs. 10%, p = 0.001). The mean gradient and SVC diameter improved from 10.8 +/- 5.8 mm Hg to 2.6 +/- 2.2 mm Hg (p < 0.001) and 3.1 +/- 2.7 mm to 9.1 +/- 3.8 mm, respectively (p < 0.001). The obstruction was adequately relieved in all 36 patients receiving stents and in 21 of 27 patients (78%) who had balloon dilation alone. Complications occurred in 12 patients (19%), all of whom had previously undergone cardiac surgery; 10 of these patients were successfully treated in the catheterization laboratory. Freedom from re-intervention did not differ between patients undergoing balloon dilation or stent implantation, but was longer in patients age >5 years at the time of intervention. CONCLUSIONS: Superior vena cava-related symptoms occur in only 50% of patients with hemodynamically significant SVC obstruction. Endovascular therapy is successful in relieving the stenosis and associated symptoms with good long-term results.

Cardiac catheterization in infants weighing less than 1,500 grams.

BACKGROUND: The improved survival of very low-birth-weight (<1,500 g) infants justifies more aggressive attempts to treat underlying congenital heart disease than in the past. METHODS: We retrospectively reviewed all catheterizations performed at our institution between January 1, 1990 and June 1, 2004 in infants weighing <1,500 g. We performed a 3:1 case-control study. Comparisons were randomly selected from a group of patients catheterized within 6 months of the cases and weighing 2-3 kg. All catheterization data, angiograms, and hospital charts were reviewed. RESULTS: Eighteen patients weighing <1,500 g underwent catheterization. Fifty-four patients were selected as comparisons. There were no significant differences in the age at catheterization, procedure time, fluoroscopy time, or contrast amount (cc/kg). The lower-birth-weight infants were more likely to be premature (median age 29 vs. 37 weeks, P < 0.001), and to have left-sided obstructive lesions including aortic stenosis or coarctation. The comparison patients were more likely to be postoperative (28% vs. 0%, P = 0.02), and included a higher number with hypoplastic left heart syndrome. There was an increased incidence of interventions performed in the lower-birth-weight infants (83% vs. 41%, P = 0.002). There was a difference in the interventions performed between the two groups: the comparisons had more atrial septal procedures, and the lower-birth-weight infants had more coarctation dilations and aortic valve dilations. There were no significant differences in the acute success rate of the procedures (100% vs. 95%), overall complication rate (56 vs. 57%), incidence of blood transfusions (44 vs. 30%), or major complications (11 vs. 13%) between the lower-birth-weight and comparison groups respectively. There was a trend towards higher survival rate in the comparison group in this small study population, but it did not reach significance (80 vs. 61%, P = 0.13). CONCLUSIONS: Cardiac catheterization in neonates <1,500 g is more likely to include percutaneous intervention, especially on the left side, but is generally successful with a complication rate similar to procedures performed in larger infants. Although these procedures are rare, improved miniaturization of equipment would facilitate safer interventions.

Conditions leading to pediatric cardiology consultation in a tertiary academic hospital.

OBJECTIVE: To determine the basis for cardiac consultations for pediatric patients in an academic hospital setting. METHODS: The activities of the cardiology consultation service were tabulated for 12 months, from July 2001 to June 2002. Patients were identified from 4 sources, ie, a monthly log of patient encounters maintained by the consultation service, encounter forms submitted to the billing office, consultation notes maintained in a central file, and a departmental list of echocardiography studies. Patients who required clearance for noncardiac surgical procedures were generally evaluated in the cardiology clinic and not by the consultation service. Patient data were obtained from consultation and echocardiography reports and from hospital computer-based records for discharge summaries for inpatient admissions, emergency department encounter summaries, and laboratory reports. For each patient, consultations were tabulated as separate encounters if they occurred on different days in the emergency department, during separate admissions, or for different clinical concerns during a single admission. RESULTS: A total of 2071 consultations were performed for 1724 patients. The age at the time of consultation was 6.6 +/- 9.3 years (median: 1.2 years; range: 1 day to 60.6 years). A total of 1507 patients (87.4%) had a single consultation; 217 patients (12.6%) had multiple encounters, ranging from 2 to 9, accounting for 564 consultations (27.2%). Clinical concerns included murmurs (18.5%), cardiac function (12.7%), arrhythmias (12.7%), intercurrent illnesses among cardiac patients (11.3%), cyanosis (6.3%), syndromes (5.7%), chest pain (5.2%), syncope/dizziness (4.5%), subacute endocarditis (4.4%), follow-up evaluations of fetal diagnoses (4.3%), Kawasaki disease (3.4%), cor pulmonale (3%), recent cardiac surgery or catheterization (1.6%), cerebrovascular accidents (1.2%), and miscellaneous conditions. Four diagnoses accounted for 91% of murmur evaluations, ie, patent ductus arteriosus, ventricular septal defects, innocent murmurs, and pulmonary branch murmur of infancy. The most common murmur diagnosis in the neonatal intensive care unit was patent ductus arteriosus (68%), in the well-child nursery was ventricular septal defect (64%), and on the medical ward was innocent murmur (62%). The most common basis for evaluation of function was oncologic disease. Among patients evaluated for function, there were 3 new diagnoses of structural congenital heart disease, all involving neonates with aortic arch obstruction. Approximately two-thirds of arrhythmias were supraventricular in origin. The most common arrhythmias requiring treatment were supraventricular tachycardia and atrial flutter/fibrillation, the latter occurring mainly among older patients with structural heart disease. Diagnoses made with fetal echocardiography accounted for 14.3% of newborn consultations and included 83% of patients with cyanotic cardiac disease. Three syndromes accounted for 57% of consultations for this indication, ie, VACTERL association (vertebral anomalies, anal atresia, congenital heart disease, tracheoesophageal fistula, renal abnormality, and limb anomalies), trisomy 21, and infant of diabetic mother. Chest pain and syncope/dizziness were frequently evaluated in the emergency department and, in this setting, accounted for 13 and 10% of all evaluations and 19 and 25% of evaluations for new patients, respectively. For patients evaluated for chest pain, the most common basis was musculoskeletal/costochondritic (42%) or idiopathic (22%). There was a cardiac or pericardial basis in 11% of cases; these patients either had known heart disease associated with this complication or systemic symptoms, abnormal cardiac auscultatory findings, and electrocardiographic features of pericarditis. Syncope/dizziness most commonly had a vasovagal (50.5%) or orthostatic (24.7%) basis. There was a cardiac basis in 5.4% of cases; these patients were more likely to have symptoms associated with exercise. Although endocarditis was a frequent clinical concern (91 patients), only 3 cases were identified, involving 2 patients with structural heart disease and 1 neonate with an indwelling intracardiac catheter. Two other patients had central venous lines, intravascular thrombus, and fungemia. Kawasaki disease was the most common acquired condition leading to consultation. Cor pulmonale was most commonly screened among patients with congenital diaphragmatic hernia, chronic lung disease of prematurity, pneumonitis, reactive airway disease, or cystic fibrosis. Patients with recent cardiac surgery or cardiac catheterization typically had postpericardiotomy syndrome or complications associated with vascular access. Approximately 20% of cases of cerebrovascular accidents had a cardiac basis. CONCLUSIONS: Although a variety of conditions were assessed, some were encountered more frequently. Future educational curricula developed for cardiac training of pediatric residents should appropriately emphasize conditions necessitating consultation.

Interventional catheterization management of perioperative peripheral pulmonary stenosis: balloon angioplasty or endovascular stenting.

There is limited reported experience of catheterization therapy for peripheral pulmonary stenosis (PPS) at a surgical site in the early postoperative period. We reviewed the clinical course of patients undergoing interventional catheterization for PPS at a surgical site < 7 weeks after surgery. Successful dilation (SD) was defined as > 50% increase in predilation diameter. From 1984 to 2000, 17 patients had 19 proximal pulmonary arteries dilated 1 to 46 (median 8) days postoperatively. Median age and weight were 3.1 year and 12.7 kg. Three arteries were initially occluded. Seventeen arteries had initial BD with postintervention imaging available in 15; 8 arteries had SD. The arterial diameter increased from 3.9 +/- 2.6 to 5.5 +/- 2.8 mm (P < 0.001). Nine of these arteries had stents placed with diameter increasing to 8.7 +/- 3.7 mm (P < 0.001 compared with post-BD diameter). Stents increased the diameter in all arteries and made four of four failed BD successful. In the two most recent procedures, stents were placed without prior BD with diameter increasing from 1.3 to 9 mm and 8.2 to 14 mm. A stent was placed in 1 of 7 arteries prior to 1993 and in 10 of 12 arteries thereafter (P < 0.004). Three patients prior to 1995 had catheterization-related deaths secondary to vessel rupture after BD. BD produces SD in approximately one-half of the procedures but is associated with mortality. Stent placement increases vessel diameter substantially more than BD alone. Stents reduce the acute complication rate and avoid early reoperation in this patient group.

Parental anxiety associated with referral of a child to a pediatric cardiologist for evaluation of a Still's murmur.

OBJECTIVE: We assessed factors contributing to parental anxiety when children are referred to a cardiology clinic for evaluation of a Still's murmur. METHODS: Parents of 95 children completed questionnaires designed to assess family and patient characteristics, parents' ratings of their anxiety and the reassurance they received from their pediatrician, and current (state) and general anxiety levels. RESULTS: Parents reported anxiety about multiple issues including the need for medication (49%), sports restrictions (41%), cardiac surgery (29%), cardiac risk for siblings (20%), and premature death (13%). Of reporting mothers, 19% felt the murmur resulted from something they did wrong during pregnancy. Although 54% of parents were extremely reassured by their pediatrician, only 17% had no anxiety associated with the specialty visit. After reassurance from the cardiologist, 7% of parents had persistent anxiety. In multivariable analysis, 2 features, both related to the referring pediatrician, were significantly related to parental anxiety level. High parental anxiety was associated with lower pediatrician reassurance ratings and greater pediatrician practice years. CONCLUSIONS: Parental anxiety is common among parents of children referred for specialty evaluation. Educational strategies to improve pediatrician communication skills with parents may improve quality of care.