Repair of Persistent Left Superior Vena Cava to Unroofed Coronary Sinus Defect by Retro-Aortic Implantation (Modified Warden Type Procedure).

BACKGROUND: Repair of unroofed coronary sinus defect in the presence of left superior vena cava is challenging and requires prosthetic graft material to redirect flow to the right atrium. This may potentially cause a supra-mitral gradient or pulmonary venous obstruction. METHODS: Three patients with unroofed coronary sinus in the presence of a left superior vena cava (LSVC) underwent modified cavo-atrial anastomosis (Warden technique) to achieve reimplantation of the LSVC in a retro-aortic fashion to the right atrial appendage. RESULTS: Three patients recovered well with no evidence of an intracardiac shunt. Postoperative echocardiography demonstrated normal venous flows in the LSVC. CONCLUSION: This modified technique offers correction of this systemic venous anomaly without the need for any additional graft material.

Interventional treatments and risk factors in patients born with hypoplastic left heart syndrome in England and Wales from 2000 to 2015.

OBJECTIVE: To describe the long-term outcomes, treatment pathways and risk factors for patients diagnosed with hypoplastic left heart syndrome (HLHS) in England and Wales. METHODS: The UK's national audit database captures every procedure undertaken for congenital heart disease and updated life status for resident patients in England and Wales. Patients with HLHS born between 2000 and 2015 were identified using codes from the International Paediatric and Congenital Cardiac Code. RESULTS: There were 976 patients with HLHS. Of these, 9.6% had a prepathway intervention, 89.5% underwent a traditional pathway of staged palliation and 6.4% of infants underwent a hybrid pathway. Patients undergoing prepathway procedures or the hybrid pathway were more complex, exhibiting higher rates of prematurity and acquired comorbidity. Prepathway intervention was associated with the highest in-hospital mortality (34.0%).44.6% of patients had an off-pathway procedure after their primary procedure, most frequently stenting or dilation of residual or recoarctation and most commonly occurring between stage 1 and stage 2.The survival rate at 1 year and 5 years was 60.7% (95% CI 57.5 to 63.7) and 56.3% (95% CI 53.0 to 59.5), respectively. Patients with an antenatal diagnosis (multivariable HR (MHR) 1.63 (95% CI 1.12 to 2.38)), low weight (<2.5 kg) (MHR 1.49 (95% CI 1.05 to 2.11)) or the presence of an acquired comorbidity (MHR 2.04 (95% CI 1.30 to 3.19)) were less likely to survive. CONCLUSION: Treatment pathways among patients with HLHS are complex and variable. It is essential that the long-term outcomes of conditions like HLHS that require serial interventions are studied to provide a fuller picture and to inform quality assurance and improvement.

The management of conjoined twins: Cardiology assessment.

Structural cardiac defects occur in at least 1 twin in about 75% of conjoined twins with thoracic level fusion. Outcomes after surgical separation of thoracic level conjoined twins have been favorable when the hearts have been separate. However, even in this situation, the outlook is poor for an individual twin with an important cardiac defect. Arterial anastomosis between twin circulations is an important additional consideration, with poor outcomes for perfusion recipient twins. Surgical separation is contraindicated when ventricular level cardiac fusion exists. Cardiac assessment is a key component of prenatal counseling.

Real-time three-dimensional fetal echocardiography--optimal imaging windows.

A total of 15 fetuses were scanned using 2-D array volumetric ultrasound (US). Acquired cardiac data were converted for rendering dynamic 3-D surface views and reformatting cross-sectional views. The image usefulness was compared between the data obtained from subcostal/subxiphoid and other imaging windows; the former are usually free of acoustic shadowing. Of 60 data sets recorded, 12 (20%) were acquired through subcostal windows in 6 (40%) patients. Subcostal windows were unavailable from the remaining patients due to unfavourable fetal positions. Of the 12 sets, 6 (50%) provided the dynamic 3-D and/or cross-sectional views of either the entire fetal heart or a great portion of it for sufficient assessments of its major structures and their spatial relationships. Of 48 data sets from other windows, only 9 (19%) provided such 3-D and/or cross-sectional views; the lower rate being due to acoustic shadowing. Real-time 3-D US is a convenient method for volumetric data acquisition. Through subcostal windows, useful information about the spatial relationships between major cardiac structures can be acquired. However, to offer detailed information, considerable improvement in imaging quality is needed.

Midterm follow-up of arterial switch operation for transposition of the great arteries with intact ventricular septum and left-ventricular outflow tract obstruction.

OBJECTIVE: We report the mid-term follow-up of patients, who underwent arterial switch operation (ASO) for transposition of the great arteries (TGA) with intact ventricular septum and left-ventricular outflow tract obstruction (LVOTO) over a 10-year period from 2000 to 2009. METHODS: Thirteen TGA patients (3.9% of our ASO cohort) with intact ventricular septum and LVOTO underwent ASO. LVOTO was defined as pulmonary valve z-score ≤ -2.0 (n=3) or peak LVOT gradient ≥40 mmHg with (n=7) or without (n=3) anatomic subvalvar stenosis on echocardiography. Median age and weight were 14 days (range, 7-130 days) and 3.2 kg (range, 2.1-4.6 kg). The LVOT abnormalities included fibromuscular narrowing (n=5) and atrioventricular valve-related findings (n=5). LVOT clearance was achieved by resection of accessory mitral tissue (n=2) only. RESULTS: Follow-up was 100% complete. There were no early or late deaths. Freedom from re-operation for neo-aortic valve regurgitation and/or LVOTO was 100% at a median follow-up of 38 months (range, 6-115 months). All patients had functional status appropriate for their age. Three patients had mild aortic regurgitation. The median Doppler estimated LVOT systolic gradient was 12 mmHg (range, 0-18 mmHg) for the entire cohort at the latest follow-up. CONCLUSIONS: Mid-term outcomes of ASO for a highly selected group of patients with pulmonary valve annulus z-score ≤ -2.0 ≥ -0.4, resectable organic LVOTO, and dynamic peak LVOT gradient ≥40 mmHg remain satisfactory, with a need for long-term follow-up.

Transcatheter closure of perimembranous ventricular septal defect: is the risk of heart block too high a price?

The late development of heart block in paediatric patients following device closure of a perimembranous ventricular septal defect may be a cause for concern.

Outcome in neonates with congenital heart disease referred for respiratory extracorporeal membrane oxygenation.

AIM: To evaluate the proportion of neonates referred for extracorporeal membrane oxygenation (ECMO) support in the modern era of advanced conventional treatments for respiratory failure who actually had congenital heart disease (CHD), and to assess the impact of this diagnostic route on patient condition and outcome. METHODS: A retrospective case-note review of neonatal ECMO and cardiac admissions to a single, tertiary ECMO and cardiac intensive care unit (ICU) between March 1999 and February 2002. RESULTS: 287 symptomatic neonates presented to the ICU with previously undiagnosed cardiac or respiratory disease. Eighty-two with presumed respiratory failure were referred for ECMO, and 205 with suspected CHD were referred for cardiac evaluation. Eight (10%) ECMO referrals, all with presumed persistent pulmonary hypertension of the newborn (PPHN), were found to have CHD (transposition: 3; total anomalous pulmonary venous connection: 3; left heart obstructive lesions: 2). Mortality in this group was 50%, compared with 11% for correctly identified CHD patients (odds ratio 8.2, 95% CI 1.92, 35.4, p<0.01). For all neonates with CHD, the risk of death was increased by the presence of cardiovascular collapse and end-organ dysfunction at presentation to the ICU (p<0.01 for both). CONCLUSION: Neonates with CHD may present as severe "PPHN" via the ECMO service. Poor outcome in these patients relates to the high incidence of cardiovascular collapse and end-organ dysfunction. Early echocardiography is recommended for neonates with presumed PPHN. Neonatal ECMO support should be based in centres with cardiac surgical services.

Late anthracycline cardiotoxicity after childhood cancer: a prospective longitudinal study.

BACKGROUND: The objective of the current study was to examine the risk factors for progression in severity of anthracycline-induced cardiac dysfunction, thereby providing information that is useful in refining cancer treatment regimes and guiding follow-up. METHODS: Serial echocardiograms were performed on 101 acute lymphoblastic leukemia survivors and 83 Wilms tumor survivors after a mean interval of 6.2 years and 6.7 years since last anthracycline dose, respectively, at first study, and after 10.3 years and 11.1 years, respectively, at second study. The paired data were contrasted with data from 100 normal subjects, and potential correlations with follow-up interval, cumulative dose, cancer diagnosis, gender, age at diagnosis, and growth were explored using univariate and multiple regression techniques. RESULTS: The most important predictor of worsening cardiac performance was total anthracycline dose. As a group, patients receiving < 240 mg/m(2) showed no deterioration of left ventricular end systolic stress at > 10 years from the end of treatment. CONCLUSIONS: Survivors who have received low-dose anthracycline require cardiac surveillance infrequently. In good prognosis tumors, cumulative anthracycline dose should be maintained at < 250 mg/m(2).

Optimal timing of the Ross procedure in the management of chronic aortic incompetence in the young.

UNLABELLED: The appropriate timing of intervention in patients with chronic aortic incompetence allows recovery of ventricular function. We sought to determine the optimal timing of the Ross procedure for chronic aortic incompetence in young patients. We retrospectively analysed case notes, and measured pre- and postoperative echocardiographic indexes of left ventricular function, in patients who had undergone the Ross procedure for chronic aortic incompetence. METHODS AND RESULTS: We found 21 patients with preoperative and postoperative data suitable for analysis. Their age at operation ranged from 5.6 to 26 years, with a median of 13.8 years, and the duration of follow-up was from 0.5 to 6.8 years, with a median of 2.4 years. The preoperative left ventricular end-diastolic dimension was converted to a z-score, and this was used as a threshold to divide the population. Using the threshold of a preoperative left ventricular z-score of more than 3 to divide the population did not show any difference in postoperative parameters of left ventricular function. Significant differences were found postoperatively, however, in both the left ventricular z-score and the ratio of left ventricular end-diastolic radius to posterior wall thickness in diastole, with a cutoff preoperative threshold z-score greater than 4. CONCLUSION: The increase in the ratio of left ventricular end-diastolic radius to the thickness of the posterior wall in diastole would suggest that there is disruption of left ventricular short axis architecture and myocardial contractile function when intervention is postponed. The significantly larger left ventricular dimension at end-diastole, despite the reduction in volume loading post surgery, may also demonstrate irreversible structural changes. Our data would suggest that recovery of left ventricular function is less likely when the left ventricular z-score has reached the value of 4, and that, ideally, intervention should be performed when the z-score approaches or exceeds 3.