Extremely premature infants born at 23-25 weeks gestation are at substantial risk for pulmonary hypertension.

OBJECTIVE: Extremely low gestational age newborns (ELGANs) represent an especially vulnerable population. Herein, we aimed to determine incidence and severity of pulmonary hypertension associated with bronchopulmonary dysplasia (BPD-PH) in extremely immature ELGANs (gestational age: 230/6-256/7 weeks). METHODS: In this prospective observational cohort study, we assessed BPD-PH by means of several echocardiography markers and serum N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels at 3 and 12 months of chronological age. In addition, we analyzed incidence and efficacy of pharmacologic treatment for BPD-PH. RESULTS: At 3 months 15/34 ELGANs had echocardiographic evidence of BPD-PH, while at 12 months of age 6/34 still had PH. PH-targeted therapy consisted of sildenafil monotherapy in 11 and dual oral combination therapy (sildenafil and macitentan) in four ELGANs at 3 and 12 months. CONCLUSION: 44% (15/34) of ELGANs developed BPD-PH. All received PH-targeted pharmacotherapy at 3 months, leading to hemodynamic improvements at 12 months in most infants.

Long-term outcomes after the paediatric Ross and Ross-Konno procedures.

OBJECTIVES: The Ross procedure is an attractive option for the management of aortic valve disease in paediatric patients. We reviewed our experience with the paediatric Ross procedure to determine survival and freedom from reoperation in the third decade after surgery. METHODS: We reviewed the data of 124 paediatric patients [71% male, median age at time of surgery 11.1 years (interquartile range 6-14.8 years); 63.7% bicuspid aortic valve], who underwent the Ross procedure at 2 tertiary centres from April 1991 to April 2020. The Ross-Konno procedures were performed on 14 (11.3%) patients. Deaths were cross-checked with the national health insurance database, and survival status was available for 96.8% of the patients. The median follow-up time was 12.1 years (interquartile range 3-18 years). RESULTS: There were 3 early and 6 late deaths. All early deaths occurred in patients aged <1 year at the time of surgery. The 25-year survival was 90.3%. Actuarial freedom from reoperation (linearized rates in parentheses) was as follows: Autograft reoperation was 90.8% (0.48%/patient-year) and right ventricular outflow tract (RVOT) reoperation was 67% (2.07%/patient year) at 25 years. The univariable Cox-proportional hazard analysis revealed younger age at time of surgery (P < 0.001), smaller implanted valve size (P < 0.001) and the use of a xenograft rather than a homograft (P < 0.001) as predictors of RVOT reoperation. At multivariable Cox-proportional hazard analysis, only age was an independent risk factor for RVOT reoperation (P = 0.041). CONCLUSIONS: The Ross and the Ross-Konno procedures are associated with good outcomes in paediatric patients. Reoperation of the RVOT is frequent and associated with younger age.

The right ventricular outflow tract in pediatric pulmonary hypertension-Data from the European Pediatric Pulmonary Vascular Disease Network.

OBJECTIVE: The right ventricular outflow tract (RVOT) is pivotal for adequate RV function and known to be adversely affected by elevated pulmonary arterial pressure (PAP) in adults with pulmonary hypertension (PH). Aim of this study was to determine the effects of increased RV pressure afterload in children with PH on RVOT size, function, and flow parameters. METHODS: We conducted a transthoracic echocardiographic study in 51 children with PH (median age: 5.3 years; range 1.5 months to 18 years) and determined the following RVOT variables: RVOT diameter, RVOT velocity time integral (VTI), ratio of tricuspid regurgitation velocity (TRV)/RVOT VTI, and RVOT systolic excursion (SE). RESULTS: In our pediatric PH cohort, the age-specific RVOT diameter z-score was higher compared to normal values. Deviation from normal RVOT diameter values increased with age, disease severity, and New York Heart Association functional class. Significant correlations were found between RVOT diameter and the RV end-diastolic area and right atrial area. The age-specific RVOT VTIz-score values were significantly lower in children with PH vs healthy controls. The TRV/RVOT VTI ratio increased with rising systolic RV pressure, while the RVOT SE was similar between PH children and control subjects. CONCLUSIONS: In pediatric PH cohort, the RVOT VTI is decreased, and the TRV/RVOT VTI ratio and the RVOT diameter increased compared to healthy subjects. Assessment of RVOT variables, together with established RV parameters, allows for a comprehensive assessment of global right heart size and performance in children with PH.

Tricuspid annular plane systolic excursion and right ventricular ejection fraction in pediatric and adolescent patients with tetralogy of Fallot, patients with atrial septal defect, and age-matched normal subjects.

AIMS: To determine whether TAPSE is an accurate marker of right ventricular (RV) systolic function in patients with tetralogy of Fallot (TOF) and patients with small atrial septal defect (ASD). The tricuspid annular plane systolic excursion (TAPSE) values were measured and compared with RV ejection fraction (EF). METHODS AND RESULTS: A prospective study was conducted in pediatric and adolescent patients with TOF (n = 110), with isolated small secundum ASD (n = 200), and age-matched patients with normally structured heart. The TAPSE values showed a positive correlation with age in both patients with ASD and normal subjects. No significant difference of TAPSE values was seen in control subjects and age-matched ASD patients. The TAPSE was not decreased compared to normal subjects in eight infant TOF patients before corrective surgery. A reduction of TAPSE values with increasing time interval following corrective surgery was seen. After a mean of 7 years TAPSE values become significantly reduced compared to age-matched controls, being below the lower bound of the -2 SD. CONCLUSION: In ASD patients the systolic RV function was preserved over the pediatric age group when compared to normal subjects. In contrast, although initially preserved, we found an impaired TAPSE with increasing postoperative period in our TOF patients.

Thrombin generation determined by calibrated automated thrombography (CAT) in pediatric patients with congenital heart disease.

INTRODUCTION: Thrombin generation was studied in pediatric patients with congenital heart disease (CHD) undergoing cardiac surgery using the calibrated automated thrombography (CAT) in terms of the lag time until the onset of thrombin formation, time to thrombin peak maximum (TTP), endogenous thrombin potential (ETP), and thrombin peak height. The possible suitability to determine the coagulation status of these patients was investigated. MATERIALS AND METHODS: CAT data of 40 patients with CHD (age range from newborn to 18 years) were compared to data using standard coagulation parameters such as prothrombin (FII), antithrombin (AT), tissue factor pathway inhibitor (TFPI), prothrombin fragment 1.2 (F 1.2), thrombin-antithrombin (TAT), activated partial thromboplastin time (aPTT), and prothrombin time (PT). RESULTS: A significant positive correlation was seen between ETP and FII (p<0.01; r=0.369), as well as between peak height and F II (p<0.01; r=0.483). A significant negative correlation was seen between ETP and TFPI values (p<0.05; r=-0.225) while no significant correlation was seen between peak height and TFPI. A significant negative correlation was seen between F 1.2 generation and ETP (p<0.05; r=-0.254) and between F 1.2 generation and peak height (p<0.05; r=-0.236). No correlation was seen between AT and ETP or peak. CONCLUSIONS: Our data indicate that CAT is a good global test reflecting procoagulatory and inhibitory factors of the hemostatic system in pediatric patients with CHD.

Aortic coarctation associated with alveolar capillary dysplasia and misalignment of the pulmonary veins.

After surgical repair of an aortic coarctation a term infant presented with severe pulmonary hypertension and cyanosis unresponsive to treatment including extracorporeal membrane oxygenation. The atypical clinical course became apparent once the accompanying diagnosis of congenital alveolar capillary dysplasia with misalignment of the pulmonary veins had been established at autopsy. In infants with congenital heart defects and with refractory pulmonary hypertension unexplainable on anatomic findings, a lung biopsy at the time of cardiac repair should be considered to avoid further therapies that would not alter the uniformly fatal course of this rare lung disorder.

Diffuse pulmonary arteriovenous malformations in a teenager with corrected total anomalous pulmonary venous drainage.

UNLABELLED: This case report describes a 13-year-old boy with diffuse microvascular pulmonary arteriovenous malformations, in whom total anomalous pulmonary venous drainage had been corrected surgically in the newborn period. Contrast transesophageal echocardiography, cardiac catheterization and a lung perfusion scan suggested, and lung biopsy confirmed the diagnosis in our patient. Treatment with nifedipin was commenced. CONCLUSION: This case illustrates the diagnostic difficulties with this rare malformation.