Clinical course of a fetus with hypoplastic left heart syndrome and premature ductal constriction.

Premature ductal constriction was diagnosed in a 30-week gestation fetus with hypoplastic left heart syndrome. The fetus developed right ventricular hypertrophy and mild tricuspid regurgitation. Foetal neuroimaging showed slowed growth of the brain and increased lactate. We describe the imaging and clinical findings. Fetuses with critical CHD and in utero ductal constriction are at increased risk of morbidity and mortality, and require appropriate counselling.

The arterial switch operation: 25-year experience with 258 patients.

BACKGROUND: At our institution, the arterial switch operation for transposition of the great arteries has transitioned from the Gore-Tex patch (W.L. Gore & Associates, Flagstaff, AZ) for pulmonary artery reconstruction to redundant pantaloon pericardial patch (RPPP). The (U-shaped) coronary artery button was used for coronary reimplantation. This study investigates overall mortality and factors for neopulmonary artery, neoaortic, and coronary artery surgical reintervention. METHODS: We performed a retrospective chart review of all patients who underwent arterial switch between 1983 and 2007. Our surgical database, operative reports, and cardiology clinic charts were reviewed. Time to event was plotted as Kaplan-Meier curves. Predictors of time-to-event were examined using Cox proportional hazard modeling. RESULTS: A total of 258 patients underwent arterial switch during the study. Mortality declined from 15% (era I: 1983 to 1990) to 11% (era II: 1991 to 1998) to 7% (era III: 1999 to 2007). Era III had a significantly later time to death compared with era I (hazard ratio [HR] 0.62, p = 0.04). The RPPP had a lower neopulmonary artery reintervention rate compared with Gore-Tex; 9 of 225 (4%) versus 3 of 21 (14%), p = 0.008. Complex anatomy increased risk for neopulmonary reintervention (HR 3.3, p = 0.03). Surgical reintervention rate for coronary arteries was 2%. Complex coronary anatomy (HR 17.9, p = 0.01) predicted coronary reintervention. Predictors of neoaortic reintervention were prior pulmonary artery band (HR 4.3, p = 0.03), complex anatomy (HR 3.5, p = 0.01), and coronary artery anatomy (HR 3.5, p = 0.04). CONCLUSIONS: Arterial switch operation mortality has decreased. Conversion to RPPP reduced neopulmonary artery reintervention. The (U-shaped) coronary artery button technique is associated with low coronary reintervention rates. Complex coronary anatomy increases coronary and aortic reintervention. Prior pulmonary artery banding and complex anatomy increase aortic reintervention.

Reoperative techniques for complications after arterial switch.

BACKGROUND: The purpose of this study is to review our experience with late reoperations after the arterial switch operation (ASO) and to introduce reparative solutions adapted from previous techniques. METHODS: A retrospective study was performed on 23 patients who underwent late reoperations after ASO between 1983 and 2010. Eighteen patients were from our concomitantly reported cohort of 258 ASO patients and 5 came from distant referrals. RESULTS: Twenty-seven reoperations on 23 patients were performed for lesions relating to coronary arteries (9 procedures, 7 patients), the neoaortic root (12 procedures, 10 patients), and the right ventricular outflow tract (6 procedures, 6 patients). Four patients died: 1 from an exsanguinating gastric ulcer 4 years after prosthetic valve replacement; 1 from coronary occlusion one month postoperatively from an unroofed intramural left main coronary artery; and 2 after supravalvar pulmonary artery stenosis repair complicated by coexisting left ventricular dysfunction from the original ASO. CONCLUSIONS: The ASO remains the treatment of choice for transposition of the great arteries and its variants. While the incidence of late reintervention is low, a subset of patients will require operations that extend the principles of myocardial revascularization, left ventricular outflow tract reconstruction, and relief of pulmonary stenosis.

Impact of telemedicine on the practice of pediatric cardiology in community hospitals.

BACKGROUND: Tele-echocardiography has the potential to bring real-time diagnoses to neonatal facilities without in-house pediatric cardiologists. Many neonates in rural areas, smaller cities, and community hospitals do not have immediate access to pediatric sonographers or echocardiogram interpretation by pediatric cardiologists. This can result in suboptimal echocardiogram quality, delay in initiation of medical intervention, unnecessary patient transport, and increased medical expenditures. Telemedicine has been used with increased frequency to improve efficiency of pediatric cardiology care in hospitals that are not served by pediatric cardiologists. Initial reports suggest that telecardiology is accurate, improves patient care, is cost-effective, enhances echocardiogram quality, and prevents unnecessary transports of neonates in locations that are not served by pediatric cardiologists. OBJECTIVE: We report the largest series to evaluate the impact of telemedicine on delivery of pediatric cardiac care in community hospitals. We hypothesized that live telemedicine guidance and interpretation of neonatal echocardiograms from community hospitals is accurate, improves patient care, enhances sonographer proficiency, allows for more efficient physician time management, increases patient referrals, and does not result in increased utilization of echocardiography. METHODS: Using desktop videoconferencing computers, pediatric cardiologists guided and interpreted pediatric echocardiograms from 2 community hospital nurseries 15 miles from a tertiary care center. Studies were transmitted in real-time using the H.320 videoconferencing protocol over 3 integrated services digital network lines (384 kilobits per second). This resulted in a frame rate of 23 to 30 frames per second. Sonographers who primarily scanned adult patients but had received additional training in echocardiography of infants performed the echocardiograms. Additional views were suggested as deemed necessary by the interpreting physician, and interpretations were made during the videoconference. The results of the echocardiogram and recommendations for patient care were communicated to the referring physician over the telemedicine system. Analyses of accuracy, patient treatment, echocardiogram quality, time to diagnosis, pediatric cardiologist practice time management, patient referral patterns, and echocardiography utilization were conducted prospectively. RESULTS: A total of 500 studies in 364 patients were transmitted during a 30-month period. The most common indication for echocardiography was to rule out congenital heart disease (208 of 500 studies). Signs and symptoms that prompted this concern included cyanosis, murmur, tachypnea, genetic syndrome, arrhythmia, abnormal fetal echocardiogram, and maternal diabetes. Other indications included suspected patent ductus arteriosus (PDA; 182 of 500 studies), intracardiac clot or catheter position, persistent pulmonary hypertension, and hemodynamic instability. Cardiac diagnoses included complex congenital heart disease (n = 16), noncritical heart disease (n = 107), and PDA (n = 86). Additional diagnoses included persistent pulmonary hypertension (n = 12), septal hypertrophy (n = 18), right atrial mass/clot/vegetation (n = 11), and decreased cardiac function (n = 6). An umbilical venous catheter was visualized in the left atrium in 9% (45 of 500) of all studies. No significant abnormalities were found in 244 studies. Major diagnoses were confirmed by subsequent review of videotape in all studies. Comparison of final videotape interpretation to initial telemedicine diagnosis resulted in 1 minor diagnostic change (membranous versus inlet ventricular septal defect). Echocardiograms were performed in subsequent visits in 264 patients. The diagnosis was altered in 3 patients. Telemedicine had an immediate impact on patient care in 151 transmissions. The most common interventions were indomethacin treatment for PDA (n = 76), retraction of umbilical venous catheters from the left atrium (n = 45), inotropic or anticongestive therapy (n = 19), anticoagulation (n = 8), and prostaglandin infusion (n = 8). Nineteen patients were transported to our hospital because of the telemedicine diagnosis. Inpatient or outpatient cardiology follow-up was recommended in an additional 131 studies and did not result in any change in the initial management. The most common diagnoses in these patients were ventricular septal defect (n = 56), atrial septal defect (n = 21), septal hypertrophy (n = 9), intracardiac thrombosis (n = 8), and pulmonary valve stenosis (n = 4). We speculate that the immediate availability of an echocardiographic diagnosis likely prevented unnecessary transport in 14 cases. Recommendations for additional views or adjustment of echocardiography machine settings were made in 95% of transmissions. Real-time guidance was especially helpful in suprasternal notch and subcostal sagittal imaging. Depth, color Doppler sector size, and color Doppler scale were frequently adjusted from routine adult settings during the teleconference. The average time from request for echocardiogram to completion of the videoconference was 28 +/- 14 minutes. This was significantly shorter than the waiting time (12 +/- 16 hours) for the videotape to be delivered by courier. Telemedicine eliminated the need for consultation in 194 cases and allowed the cardiologist to delay the visit until the end of the day in an additional 26 cases. This resulted in average time savings of 4.2 person-hours/wk based on travel and consultation time. Utilization of echocardiography was similar before (35 of 1000 births) and after (33 of 1000 to 43 of 1000) telemedicine installation. The percentage of neonatal echocardiograms that were interpreted by our practice increased from 63% to 81% at 1 hospital and from 0% to 100% at the other hospital. CONCLUSION: Real-time transmission of neonatal echocardiograms from community hospitals over 3 integrated services digital network lines is accurate and has the potential to improve patient care, enhance echocardiogram quality, aid sonographer education, and have a positive impact on referral patterns and time management without increasing the utilization of echocardiography.