Repair of isolated atrial septal defect in infants less than 12 months improves symptoms of chronic lung disease or shunt-related pulmonary hypertension.

INTRODUCTION: Infants with isolated atrial septal defects are usually minimally symptomatic, and repair is typically performed after infancy. Early repair may be considered if there is high pulmonary blood flow and reduced respiratory reserve or early signs of pulmonary hypertension. Our aim was to review the characteristics and outcomes of a cohort of patients who underwent infant repair at our institute. METHODS: The study included 56 infants (28 female, 19 trisomy 21) with isolated atrial septal defect (age: 8 months (1.5-12), weight: 6 kg (2.8-7.5), echo Qp/Qs: 1.9 ± 0.1) who underwent surgical closure (20 fenestrated). Three groups were identified: 1) chronic lung disease and pulmonary hypertension (group A: n = 28%); 2) acutely unwell infants with pulmonary hypertension but no chronic lung disease (group B: n = 20, 36%); and 3) infants with refractory congestive heart failure without either pulmonary hypertension or chronic lung disease (group C: n = 9, 16%). RESULTS: Post-operatively, pulmonary hypertension infants (47/56) showed improvement in tricuspid annular plane systolic excursion z-score (p < 0.001) and right ventricular systolic/diastolic duration ratio (p < 0.05). All ventilator (14.3%) or oxygen-dependent (31.6%) infants could be weaned within 2 weeks after repair. One year later, weight z-score increased in all patients and by +1 in group A, +1.3 in group B and +2 in group C. Over a median follow-up of 1.4 years, three patients died, four patients continued to have pulmonary hypertension evidence and two remained on targeted pulmonary hypertension therapy. CONCLUSION: Atrial septal defect repair within the first year may improve the clinical status and growth in infants with early signs of pulmonary hypertension or those requiring respiratory support and facilitate respiratory management.

Value of tissue Doppler echocardiography in children with pulmonary hypertension.

BACKGROUND: The impact of pulmonary hypertension (PHT) on right ventricular and left ventricular (LV) function in children with PHT is unknown, and echocardiographic data combining conventional and Doppler tissue imaging (DTI) on PHT in children are sparse. METHODS: Forty-one children (18 male; mean age, 7.9 ± 5.6 years) with PHT and structurally normal hearts (27 with idiopathic PHT, 14 with associated PHT) and 44 age-matched healthy controls were assessed using conventional echocardiography and DTI. RESULTS: Children with PHT had enlarged tricuspid valve diameters, right atrial areas, pulmonary artery dimensions, and LV eccentricity indices. In addition, pulmonary acceleration time and tricuspid annular plane systolic excursion were significantly reduced in patients compared with controls. DTI revealed that children with PHT had significantly lower systolic (S) and early diastolic (E) velocities at the tricuspid and septal levels. Despite preserved LV ejection fractions, left lateral free wall systolic velocities were significantly reduced in patients with PHT. Significantly reduced LV rapid filling velocities (E) suggested an underloaded left ventricle or LV diastolic dysfunction in children with PHT compared with controls. Pulmonary acceleration time and tricuspid annular plane systolic excursion correlated best with DTI systolic tricuspid and septal velocities. CONCLUSIONS: Despite not being evident on conventional two-dimensional echocardiography, LV systolic performance appears to be impaired in children with PHT. Quantitative DTI assessment of ventricular function and ventricular-ventricular interactions in this setting might provide further insights into the mechanisms leading to end-stage PHT and may guide clinicians to optimize antifailure treatment.

Subcostal real-time three-dimensional echocardiography of interatrial communications: reconstruction of an oval fossa defect, a superior sinus venosus defect with partially anomalous pulmonary venous drainage, an infero-posterior oval fossa defect, and a coronary sinus defect.

UNLABELLED: Real-time three-dimensional echocardiography can surpass simple cross-sectional echocardiography in providing precise details of cardiac lesions. For the purpose of optimising treatment, we describe our findings with real-time three-dimensional echocardiography when interrogating different types of communications permitting interatrial shunting. A three-dimensional reconstruction of defects within the oval fossa enabled reliable identification of location, size, and integrity of surrounding rims. In the superior sinus venosus defect associated with partially anomalous pulmonary venous drainage, three-dimensional reconstruction helped to provide a better understanding of the relationship between the interatrial communication, the orifice of the superior caval vein, and the connections of the right upper pulmonary vein. In the defect opening infero-posteriorly within the oval fossa, three-dimensional reconstruction helped to avoid the risk of potentially inappropriate closure of the defect by suturing the hyperplastic Eustachian valve to the atrial wall, which could have diverted the inferior caval venous return into the left atrium, or obstructed the caval venous orifice. In the coronary sinus defect, three-dimensional echocardiography provided a 'face to face' view of the entire coronary sinus roof, showing a circular defect communicating with the cavity of the left atrium. Acquisition of the full-volume data sets took less than 2 minutes for the patients having defects within the oval fossa, and no more than 3 minutes for the patients with the sinus venosus and coronary sinus defects. Post-processing for the defects in the oval fossa took from 5 to 8 minutes, and from 12 to 16 minutes for the more complicated defects. CONCLUSION: Cross-sectional two-dimensional echocardiography can establish correct diagnosis in all types of atrial communications; however, real-time three-dimensional reconstruction provides additional value to the surgeon and interventionist for better understanding of spatial intracardiac morphology.

The clinical utility of brain natriuretic peptide in paediatric left ventricular failure.

AIMS: To assess the role of brain natriuretic peptide (BNP) in both the acute and chronic settings in children with left ventricular (LV) failure. METHODS AND RESULTS: We undertook a retrospective review of all BNP levels taken over a 2-year period in our institution. Minimum follow-up was 90 days. Ninety-two BNP samples from 48 patients were reviewed. Twenty patients (42%) reached the combined endpoint of death, transplantation, or listing for transplantation. Median age was 3 years and 3 months. Mean BNP levels in NYHA or Ross classes I-IV were 29, 239, 744, and 1593 pg/mL, respectively, with significant differences between mean logBNP in classes I-III (P < 0.001). LogBNP levels correlated with fractional shortening (P < 0.001), LVEDd z-score (P < 0.001), and tissue Doppler velocities (P < 0.02). From serial data there was a strong correlation between change in BNP and change in clinical status (F 9.5, P < 0.001). Receiver-operator curve (ROC) demonstrated that BNP > 290 pg/mL predicts poor outcome with sensitivity of 0.80, specificity of 0.87, and likelihood ratio of 6.4 in paediatric patients with chronic LV dysfunction. A separate ROC from acute presentations did not demonstrate superiority of BNP over other assessments. CONCLUSION: BNP levels in paediatric heart failure (HF) patients show a strong correlation to both impaired heart function on echocardiogram and clinical status. Serial BNP levels follow the clinical course. In chronic HF, a BNP level of >290 pg/mL is predictive of an adverse outcome.