A 16 year old girl underwent percutaneous balloon dilation of the pulmonary valve. The procedure was carried out in the catheterization laboratory under transesophageal echocardiographic guidance with the patient anesthetized (ketamine) without endotracheal intubation. A 64 elements phased array "omniplane" TEE probe connected to a Sonos 1500 Hewlett-Packard system was used. The transducer assembly encased within the tip of the endoscope can be rotated through a 180 degrees arc counterclockwise and clockwise, thus obtaining a multitude of imaging planes. The right ventricular outflow tract and pulmonary valve could be visualized very well and therefore monitored accurately during all phases of the procedure. In the future this technique will be able to reduce the need for X-ray exposure and dye injection during percutaneous pulmonary valvuloplasty especially in cases in which their use could be contraindicated.
The approach to the diagnosis of congenital heart disease, based upon the identification of the cardiac chambers, the reconstruction of their sequence and final recognition of the basic circulatory model is discussed. Three cardiac segments are recognizable embriologically, anatomically and functionally: atria, ventricles and great arteries. Connexion is the sequential link of these segments, independently from their spatial relationship. Situs of the atria can be: solitus, inversus or ambiguus, and is determined by the thoracic situs which can be assumed from the bronchial anatomy. Atrio-ventricular connexion can be concordant, absent or double inlet ventricle. Ventricular-arterial connexion can be concordant, discordant, double outlet ventricle or single outlet heart. Since this approach aims to the reconstruction of the connexion among the cardiac segments, heart position and the infundibular anatomy are not relevant to the recognition of the circulatory model.
To the Editor.—Thangaroopan et al1 recently reported that pediatric cardiac assessment (CA) provided incremental diagnostic yield in comparison with fetal echocardiography (FE) in the offspring of women with congenital heart disease (CHD). We agree with the important role of CA in managing these patients. However, 2 aspects should be considered in interpreting these findings. First, caution is required when considering as effectively incremental the diagnostic information provided by a test in comparison with another when the 2 tests are not performed independently and in a blinded fashion. In particular, both in research and clinical practice, the risk that interpretation of CA findings may be affected by knowledge of FE results should be taken into account. In the presence of positive FE findings, physicians may be encouraged to assess CA features with particular attention and emphasize borderline findings; in contrast, because of the well-known low sensitivity of FE in detecting noncomplex lesions,2 a negative FE finding usually does not affect interpretation of CA findings significantly. In this study, this concept is highlighted by the very low prevalence of children with positive FE and negative CA findings (2.2%) compared with those with negative FE and positive CA findings (12.7%). As a result of this bias, the number of children with CHD missed by CA is reduced, thus leading to a spurious increase in sensitivity.Second, Thangaroopan et al stressed that CA allowed correct detection of CHD in 18 (6.5%) infants in whom diagnosis had been missed by FE. However, the presence of 17 (6.2%) normal infants without CHD correctly identified by FE (as confirmed by subsequent pediatric echocardiography) and in whom CA findings were erroneously positive should also be pointed out. Unfortunately, because pediatric echocardiography was not performed on all patients, the sensitivity and specificity of FE and CA cannot be calculated. Nonetheless, assuming that infants with concordant positive results between FE and CA were effectively affected by CHD, and those with concordant negative results were normal, it could be estimated that specificity of FE alone was 97.6% (248 of 254 normal patients correctly identified), whereas specificity of the approach combining FE and postnatal CA, as proposed by the authors, was 90.9% (231 of 254 normal patients correctly identified).On the basis of these considerations, and taking into account that additional lesions detected by CA and missed by FE were all minor or noncomplex CHD, the opportunity to routinely perform FE followed by postnatal CA in the offspring of women with CHD should be critically considered.
Abstract The aim of this single‐centre retrospective observational study was to evaluate the safety, tolerability, and efficacy of an in‐class combination therapy switch from bosentan plus sildenafil to ambrisentan plus tadalafil in children with pulmonary arterial hypertension. Children aged over 5 years who were established on sildenafil plus bosentan were offered to undergo a therapy switch from May 2014 to May 2021 and, if remaining in the service, followed up to May 2024. Children with Eisenmenger syndrome, open intra or extra‐cardiac shunt, or with pulmonary hypertension‐associated lung disease were excluded. As part of a structured clinical program children were assessed via walk test, echocardiography, cardiac magnetic resonance imaging (CMRI), cardiopulmonary exercise testing, and serum biomarkers. Fifty‐two children were included, 33 in the switch group and 19 in the control group. Clinical characteristics at diagnosis and baseline assessments did not differ between groups. All children tolerated the medication switch. Over a median 13.0 [12.0,13.7] week follow‐up in the switch group there was a significant improvement in World Health Organization functional class (WHO FC, p < 0.001); reduction in estimated right ventricular systolic pressure by echocardiography of 7 mmHg ( p = 0.03) and a 2% increase ( p = 0.03) in right ventricular ejection fraction on CMRI. There was a sustained improvement in WHO FC ( p < 0.01) in the switch group at medium‐term follow‐up of 40.9 [35.2,49.3] weeks. Long‐term outcome of transplant‐ or Potts shunt‐free survival was comparable between the two groups.