Early changes in ventricular septal defect size and ventricular geometry in the single left ventricle after volume-unloading surgery

Objectives.This study investigated the phenomenon of, and the relation between, alterations in ventricular geometry after acute surgical volume unloading of the ventricle and the development of subaortic stenosis in patients with a single ventricle and ventricular septal defect—dependent systemic flow.Background.Subaortic outflow obstruction has been observed to occur in patients with a single left ventricle after placement of a pulmonary artery band. The timing and etiology of this phenomenon are not well defined.Methods.The preoperative and postoperative echocardiograms of 18 patients 14.9 ± 22.8 months old (mean ± SD) with a diagnosis of single left ventricle who underwent pulmonary artery banding or cavopulmonary connection were reviewed. Postoperative studies were performed a mean of 7.0 ± 6.5 days after operation. The ventricular septal defect diameter was measured in two orthogonal views and the area calculated using the formula for an ellipse. Interventricular septal and posterior wall thickness and left ventricular diameter and length were also measured.Results.Mean ventricular septal defect area indexed to body surface area diminished by 36 ± 23% (3.1 ± 2.7 to 2.0 ± 1.8 cm2/m2, p < 0.01). Mean interventricular septal and posterior wall thickness increased significantly, and left ventricular diameter and length decreased significantly. A greater diminution in ventricular septal defect area was noted after cavopulmonary connection (41 ± 19%, p < 0.01) than after pulmonary artery banding (25 ± 28%, p = 0.22).Conclusions.In the single left ventricle, diminution in ventricular septal defect size occurs early and is related to an acute alteration in ventricular geometry that accompanies the decrease in ventricular volume. Ventricular septal defect diminution was greater after volume unloading of the ventricle after cavopulmonary connection than after pulmonary artery banding

Factors influencing efficacy and satisfaction in prenatal counseling for families with single ventricle heart disease

Introduction: The Fetal Heart Program at Children’s Hospital of Philadelphia offers prenatal counseling to families with congenital heart disease diagnoses. This center has yet to examine family satisfaction with counseling, especially those with complicated single ventricle diagnoses. While families may receive sufficient education about their child’s condition, better communication may be needed to prepare them for potential neurological and behavioral complications. Methods: Through chart review, 355 patient families with single ventricle diagnoses who utilized CHOP prenatal counseling within the past 10 years were identified. Retrospective surveys including Likert scales and open-ended questions were distributed by email. 65 responses were received. Average satisfaction scores with members of the clinical team and average levels of preparedness for aspects of clinical care were calculated. Results: Analysis is ongoing. Initial findings revealed high levels of satisfaction with the clinical team. High levels of preparedness were seen in all areas except for neurological and behavioral complications. Free responses indicated overall gratefulness for the counseling and familial resilience despite challenging clinical courses. Discussion: Single ventricle heart disease requires significant surgical intervention and places extreme stress on families. CHOP prenatal counseling provides families with comprehensive education about their child’s condition but fails to fully prepare them for potential neurological and behavioral problems. Additional support is needed to assist families in navigating these complications and determine risk factors for their development

Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection

AbstractBackground and methods: Between January 1, 1984, and December 1, 1997, 73 infants with functional single ventricle and total anomalous pulmonary venous connection were admitted to our institution. A retrospective review was undertaken to determine factors influencing survival. Results: Heterotaxy syndrome was present in 52 patients and hypoplastic left heart syndrome in 14. Obstructed total anomalous pulmonary venous connection was present in 21 patients. The pulmonary venous connection was supracardiac in 32 patients, cardiac in 21 patients, infracardiac in 13, and mixed in 7. Twelve patients died before the operation. The remaining 61 patients underwent surgery at a median age of 5 days (range 1 day–2.5 years). Overall survival was 45% at 6 months of age, 37% at 1 year, and 19% at 5 years. Survival for patients undergoing surgery was 54% at 6 months of age, 44% at 1 year, and 23% at 5 years. By univariate analysis with the Cox proportional hazards model, younger age at the time of the initial operation and repair of total anomalous pulmonary venous connection were predictors of mortality. Lung tissue from 14 patients was available for histologic examination. The pulmonary veins were dilated and wall thickness was increased. Increased muscularization of the arteries was seen in 11 patients. Conclusions: The long-term prognosis for children undergoing staged reconstructive operations for single ventricle and total anomalous pulmonary venous connection is poor. Early mortality is high and late death is a continuing risk. Development of the pulmonary vasculature, especially the pulmonary veins, is abnormal, even in children without clinical evidence of pulmonary venous obstruction. (J Thorac Cardiovasc Surg 1999;117:506-14

Eleven fetal echocardiographic planes using 4-dimensional ultrasound with spatio-temporal image correlation (STIC): a logical approach to fetal heart volume analysis

<p>Abstract</p> <p>Background</p> <p>Theoretically, a cross-sectional image of any cardiac planes can be obtained from a STIC fetal heart volume dataset. We described a method to display 11 fetal echocardiographic planes from STIC volumes.</p> <p>Methods</p> <p>Fetal heart volume datasets were acquired by transverse acquisition from 200 normal fetuses at 15 to 40 weeks of gestation. Analysis of the volume datasets using the described technique to display 11 echocardiographic planes in the multiplanar display mode were performed offline.</p> <p>Results</p> <p>Volume datasets from 18 fetuses were excluded due to poor image resolution. The mean visualization rates for all echocardiographic planes at 15-17, 18-22, 23-27, 28-32 and 33-40 weeks of gestation fetuses were 85.6% (range 45.2-96.8%, N = 31), 92.9% (range 64.0-100%, N = 64), 93.4% (range 51.4-100%, N = 37), 88.7%(range 54.5-100%, N = 33) and 81.8% (range 23.5-100%, N = 17) respectively.</p> <p>Conclusions</p> <p>Overall, the applied technique can favorably display the pertinent echocardiographic planes. Description of the presented method provides a logical approach to explore the fetal heart volumes.</p

Surgical Management of Left Ventricular Outflow Tract Obstruction after Biventricular Repair of Double Outlet Right Ventricle

Regardless of the preoperative morphology and the type of operation, left ventricular outflow tract obstruction (LVOTO) after biventricular repair of double outlet right ventricle (DORV) may develop. This report presents our 10-yr experience with surgical management of LVOTO after biventricular repair of DORV. Between 1996 and 2006, 15 patients underwent reoperation for subaortic stenosis after biventricular repair of DORV. The mean age at biventricular repair was 23.3±18.3 months (1.1-64.2). Biventricular repairs included tunnel constructions from the left ventricle to the aorta in 14 cases and an arterial switch operation in one. The mean left ventricle-to-aorta peak pressure gradient was 54.0±37.7 mmHg (15-140) after a mean follow-up of 9.5±6.3 yr. We performed extended septoplasty in nine patients and fibromuscular resection in six. There were no early or late mortality. There was one heart block and one aortic valve injury after an extended septoplasty, and two and one after a fibromuscular resection. No patient required reoperation for recurrent subaortic stenosis. The mean pressure gradient was 11.2±11.4 mmHg (0-34) after a mean follow-up of 5.6±2.7 yr. Extended septoplasty is a safe and effective method for the treatment of subaortic stenosis, especially in cases with a long-tunnel shaped LVOTO

Degree of twisting and duration of symptoms are prognostic factors of testis salvage during episodes of testicular torsion

Background: Testicular torsion is surgical emergency. Prompt diagnosis and treatment of testicular torsion is essential for testicular viability. At surgical exploration, the spermatic cord is seen twisted a variable number of times around its longitudinal axis. There is scant data regarding the degree of twisting and its association with testis outcomes. The purpose of our study is to explore how the degree of torsion factors into testicular outcome using follow-up data. Methods: We retrospectively reviewed the records of adolescent males who presented with testicular torsion to our institution, looking at duration of pain symptoms, degree of torsion documented in the operative note, procedure performed (orchiopexy versus orchiectomy), and follow-up clinic data for whether testicular atrophy after orchiopexy was present. A non-salvageable testis was defined as orchiectomy or atrophy. Receiver operator characteristics (ROC), multivariate, and logistic regression analyses were performed to determine the probability of a non-salvageable torsed testis based on time and degree of twisting. Results: Eighty-one patients met our study criteria, with 55 testes deemed viable and 26 non-salvageable. We found a 25.7% atrophy rate after orchiopexy. Cut-off values of 8.5 h and 495 degrees of torsion would provide sensitivities of 73% and 53%, respectively, with specificity of 80% for both. Only duration and age were correlated with the risk of non-salvage on multivariate analysis. Logistic regression generated linear probability formulas of 4 + (3 × hours) and 7 + (0.05 × degrees) in calculating the probability of non-salvage with strong correlation. Conclusions: We were able to derive separate formulas to determine the viability of the torsed testis based on symptom duration and degrees of twisting. Fifteen h of symptoms and 860 degrees of torsion gives testes a 50% salvage rate. Interestingly, we also found that about 1 out of every 4 testes undergoes atrophy after orchiopexy

Survival Data and Predictors of Functional Outcome an Average of 15 Years after the Fontan Procedure: The Pediatric Heart Network Fontan Cohort

ObjectiveMulticenter longitudinal outcome data for Fontan patients surviving into adulthood are lacking. The aim of this study was to better understand contemporary outcomes in Fontan survivors by collecting follow‐up data in a previously well‐characterized cohort.DesignBaseline data from the Fontan Cross‐Sectional Study (Fontan 1) were previously obtained in 546 Fontan survivors aged 11.9 ± 3.4 years. We assessed current transplant‐free survival status in all subjects 6.8 ± 0.4 years after the Fontan 1 study. Anatomic, clinical, and surgical data were collected along with socioeconomic status and access to health care.ResultsThirty subjects (5%) died or underwent transplantation since Fontan 1. Subjects with both an elevated (>21 pg/mL) brain natriuretic peptide and a low Child Health Questionnaire physical summary score (<44) measured at Fontan 1 were significantly more likely to die or undergo transplant than the remainder, with a hazard ratio of 6.2 (2.9–13.5). Among 516 Fontan survivors, 427 (83%) enrolled in this follow‐up study (Fontan 2) at 18.4 ± 3.4 years of age. Although mean scores on functional health status questionnaires were lower than the general population, individual scores were within the normal range in 78% and 88% of subjects for the Child Health Questionnaire physical and psychosocial summary score, and 97% and 91% for the SF‐36 physical and mental aggregate score, respectively. Since Fontan surgery, 119 (28%) had additional cardiac surgery; 55% of these (n = 66) in the interim between Fontan 1 and Fontan 2. A catheter intervention occurred in 242 (57%); 32% of these (n = 78) after Fontan 1. Arrhythmia requiring treatment developed in 118 (28%) after Fontan surgery; 58% of these (n = 68) since Fontan 1.ConclusionsWe found 95% interim transplant‐free survival for Fontan survivors over an average of 7 years of follow‐up. Continued longitudinal investigation into adulthood is necessary to better understand the determinants of long‐term outcomes and to improve functional health status.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/110738/1/chd12193.pd

Disproportionate cardiac hypertrophy during early postnatal development in infants born preterm.

Background Adults born very preterm have increased cardiac mass and reduced function. We investigated whether a hypertrophic phenomenon occurs in later preterm infants and when this occurs during early development. Methods Cardiac ultrasound was performed on 392 infants (33% preterm at mean gestation 34±2 weeks). Scans were performed during fetal development in 137, at birth and 3 months of postnatal age in 200, and during both fetal and postnatal development in 55. Cardiac morphology and function was quantified and computational models created to identify geometric changes. Results At birth, preterm offspring had reduced cardiac mass and volume relative to body size with a more globular heart. By 3 months, ventricular shape had normalized but both left and right ventricular mass relative to body size were significantly higher than expected for postmenstrual age (left 57.8±41.9 vs. 27.3±29.4%, P<0.001; right 39.3±38.1 vs. 16.6±40.8, P=0.002). Greater changes were associated with lower gestational age at birth (left P<0.001; right P=0.001). Conclusion Preterm offspring, including those born in late gestation, have a disproportionate increase in ventricular mass from birth up to 3 months of postnatal age. These differences were not present before birth. Early postnatal development may provide a window for interventions relevant to long-term cardiovascular health