Rachel E. Andrews

New-Onset Heart Failure Due to Heart Muscle Disease in Childhood A Prospective Study in the United Kingdom and Ireland

Background— We undertook the first prospective, national, multicenter study to describe the incidence and outcome of heart muscle disease–induced heart failure in children. Methods and Results— Data were collected on patients admitted to a hospital through 2003 with a first episode of heart failure in the absence of congenital heart disease. All 17 pediatric cardiac centers in the United Kingdom and Ireland participated. Follow-up data were obtained to a minimum of 1 year. The incidence was 0.87/100 000 population <16 years (n=104; 53 girls; 95% confidence interval 0.71 to 1.05 per 100 000). Median age at presentation was 1 year, with 82% in New York Heart Association class III to IV. Causes of heart failure included dilated cardiomyopathy (50 idiopathic, 8 familial), probable myocarditis (23), occult arrhythmia (7), anthracycline toxicity (5), metabolic disease (4), left ventricular noncompaction (3), and other (4). Overall 1-year survival was 82%, and event (death or transplantation)-free survival was 66%. Regression analysis showed older age and reduced systolic function on admission echocardiogram increased the event risk. Only 8% of event-free survivors (n=69) remained in New York Heart Association class III to IV, but 35 required readmission during the study period, and all but 8 remained on medication. Conclusions— This first national prospective study of new-onset heart failure in children has shown an incidence of 0.87/100 000. Multivariable analysis of survival data indicates a better outcome for younger children and for those with better systolic function at presentation, but overall, one third of children die or require transplantation within 1 year of presentation.

Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis

BACKGROUND AND AIMS Staged reconstructive surgery has radically altered the prognosis of hypoplastic left heart syndrome (HLHS). Antenatal diagnosis allows for appropriate counselling, and time to consider treatment options. We report outcome from a centre where most cases are antenatally diagnosed and delivered on site. METHODS Information was collated on 188 consecutive cases of HLHS between 1995 and 2000, including timing of diagnosis, outcome of pregnancy, and age and outcome at each stage of surgery. At Guys Hospital, 174 cases were diagnosed antenatally, of whom 50 underwent surgery. Fourteen others (five diagnosed antenatally at other centres, and nine diagnosed postnatally) also underwent surgery. RESULTS Survival after stage I (the Norwood operation) was 52% (33/64). Postoperative survival after stage II (the hemi-Fontan operation, performed in 29), and stage III (the Fontan operation, performed in 10), was 100%. Two late deaths occurred 3 and 10 months after stage II, giving overall survival of 48% (31/64). At follow up, three children have neurological impairment, and one had poor right ventricular function necessitating cardiac transplantation. CONCLUSIONS Antenatal diagnosis allows informed decisions about treatment options, and facilitates preoperative care. Mortality following stage I is high, irrespective of timing of diagnosis, but medium term outcome for survivors is good.

Peak Oxygen Uptake Correlates With Survival Without Clinical Deterioration in Ambulatory Children With Dilated Cardiomyopathy

Background— Children stable at home with dilated cardiomyopathy remain at risk of death; there is evidence of survival benefit for transplantation out to 4 years postoperatively. The limited supply of donor organs makes risk stratification imperative, but although cardiopulmonary exercise test is well established as a powerful tool in adults with heart failure, no published studies have linked oxygen uptake to prognosis in children. Methods and Results— Between 2001 and 2009, using cardiopulmonary exercise test and echocardiography, we studied 82 children (mean age, 13.5±2.3 years) with dilated cardiomyopathy. All were ambulatory, outpatients, and >120 cm in height. All children completed a symptom-limited maximal exercise test. Resting left ventricular shortening fraction was 20±9%; peak heart rate was 87±13% of predicted; peak oxygen uptake (V[Combining Dot Above]O2) was 67±22% of predicted; and ventilatory efficiency was 32±8. Follow-up was available for 100% of the children, and was a mean of 32.3±7.5 months. Eighteen patients reached the defined clinical end point of death or listing for urgent heart transplantation. On univariate analysis, left ventricular shortening fraction, peak heart rate, peak V[Combining Dot Above]O2, peak systolic blood pressure, and ventilatory efficiency were all associated with adverse outcome. On multivariable Cox analysis, only peak V[Combining Dot Above]O2 (P=0.003) was associated with the study end point. Patients with a peak V[Combining Dot Above]O2 ⩽62% of predicted had a higher 24-month event rate (50.6% versus 4.4%; hazard ratio, 10.78). Conclusions— We have demonstrated that a cardiopulmonary exercise test is feasible in ambulatory children with dilated cardiomyopathy who are >120 cm height and for the first time have linked peak V[Combining Dot Above]O2 with outcome in children.

Correlation of maternal flecainide concentrations and therapeutic effect in fetal supraventricular tachycardia

BACKGROUND Transplacental flecainide is an established therapy for fetal supraventricular tachycardia (SVT), but there is a paucity of data regarding the dose-response relationship. OBJECTIVE The purpose of this study was to investigate the relationship between maternal flecainide concentrations, arrhythmia control, and adverse fetal effects in fetal SVT. METHODS Fetuses with SVT treated with transplacental flecainide at our tertiary fetal cardiology unit between 1997 and 2012 were retrospectively studied. The maternal trough flecainide concentrations throughout treatment were collated, and clinical notes were reviewed to ascertain the response to therapy and fetal outcome. RESULTS Thirty-three fetuses were treated at a median (range) gestation of 28 weeks (20-38 weeks). Median fetal heart rate was 250/min (range 207-316/min). One patient was lost to follow-up, and this fetus was excluded from further analysis. In total, 25 of 32 fetuses (78%) converted to sinus rhythm. Median time to conversion to sinus rhythm was 3 days (range 2-12 days). Median flecainide concentration was 460 μg/L (range 250-866 μg/L) at conversion to sinus rhythm. Flecainide concentrations were not significantly different between responders and nonresponders (P = .849). Twelve of 14 hydropic and 13 of 18 nonhydropic fetuses converted to sinus rhythm with similar flecainide concentrations (P = .316). No fetus achieved cardioversion with a maternal serum flecainide concentration <250 μg/L. No fetus died while being treated with flecainide. CONCLUSION The clinical response to flecainide appears good, even in hydropic fetuses. Trough maternal flecainide concentrations, once therapeutic, do not predict cardioversion in the fetus with SVT. Flecainide therapy appears both safe and effective for the fetus when monitored appropriately.

Thoracoscopic Pericardiectomy for Constrictive Pericarditis in a Pediatric Patient With Mulibrey Nanism

Mulibrey nanism is a rare autosomal recessive disease with multisystem involvement. Clinical deterioration is most often related to cardiac involvement in the form of restrictive or constrictive disorders, and pericardiectomy may be required. We report a case of Mulibrey nanism in a patient of non-Finnish origin, where a thoracoscopic pericardiectomy helped in good palliation and clinical recovery.

Response to Letter Regarding Article, “Peak Oxygen Uptake Correlates With Survival Without Clinical Deterioration in Ambulatory Children With Dilated Cardiomyopathy”

We would like to thank Dr Singh for his letter and kind comments regarding our article recently published in Circulation .1 Our article, which included 82 children with dilated cardiomyopathy who underwent a cardiopulmonary exercise test as outpatients, found a strong association between peak oxygen uptake (Vo2) and the subsequent risk of dying or being urgently listed for transplantation. Dr Singh has highlighted that the design of our study favored a larger effect size than that previously observed by Mancini et al2 in adults on the …

Response to Letter Regarding Article, “New-Onset Heart Failure Due to Heart Muscle Disease in Childhood: A Prospective Study in the United Kingdom and Ireland”

We thank Dr Sane for his comments on our article. We agree that vitamin D deficiency is an underdiagnosed cause of cardiomyopathy in infants. However, our documentation of vitamin D–associated cardiomyopathy in this series is noteworthy because the recent large North American series of >900 cases of pediatric cardiomyopathy1 did not reveal any cases. It is likely that screening for this …

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.