Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension concomitant with Klippel-Trenaunay-Weber syndrome.

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital disorder characterized by cutaneous capillary malformations, bone hypertrophy, and multiple venous or lymphatic malformations. KTWS is associated with chronic thromboembolic pulmonary hypertension (CTEPH), presumably due to thromboembolism from multiple vascular malformations. Here, we report the first case series of patients with KTWS-CTEPH who underwent balloon pulmonary angioplasty (BPA). Both patients are alive 20 years and 1 year after the initial diagnosis of CTEPH, respectively, and are stable with improved hemodynamics. BPA may be an effective treatment option for patients with KTWS-CTEPH.

Growth recovery lines in children: A comparison between psychosocial short stature and other pathological causes of short stature.

BACKGROUND: Psychosocial short stature (PSS) is a rare disorder associated with emotional deprivation. Growth recovery lines (GRLs), the radiodense bands in metaphyseal bones, are indicators of stress. OBJECTIVE: To evaluate the efficacy of using GRLs in the distal radius to identify PSS. PARTICIPANTS AND SETTING: This retrospective cohort study included children 15-138 months of age with short stature whose hands and wrists were radiographed between 2011 and 2020 at Matsuyama Red Cross Hospital in Japan. METHODS: PSS was determined if a child with short stature had been reported to be abused or neglected. Other pathological short statures were diagnosed per the established criteria. GRLs, height velocity before and after specific treatment, insulin-like growth factor 1, and the difference between chronological and skeletal age were assessed. RESULTS: The PSS and other short stature groups comprised of 7 and 11 children, respectively. The body mass index of the PSS group was smaller than that of other short stature group (-1.15 standard deviation [SD] vs. 0.57 SD, P = 0.003). The PSS group had significantly more GRLs than the other group (5.3 vs. 0.5, P = 0.011). Height velocity before treatment in the PSS group was significantly lower (-5.46 SD vs. -1.86 SD, P = 0.005), with no significant differences in other variables. The specificity for PSS was >90% when children with short stature had at least three GRLs in both distal radii. CONCLUSIONS: Abuse or neglect should be considered in children with short stature having multiple GRLs in the distal radius.

High Right Ventricular Afterload during Exercise in Patients with Pulmonary Arterial Hypertension.

The right ventricle (RV) is more sensitive to an increase in afterload than the left ventricle (LV), and RV afterload during exercise increases more easily than LV afterload. Pulmonary arterial hypertension (PAH)-specific therapy has improved pulmonary hemodynamics at rest; however, the pulmonary hemodynamic response to exercise is still abnormal in most patients with PAH. In these patients, RV afterload during exercise could be higher, resulting in a greater increase in RV wall stress. Recently, an increasing number of studies have indicated the short-term efficacy of exercise training. However, considering the potential risk of promoting myocardial maladaptive remodeling, even low-intensity repetitive exercise training could lead to long-term clinical deterioration. Further studies investigating the long-term effects on the RV and pulmonary vasculature are warranted. Although the indications for exercise training for patients with PAH have been expanding, exercise training may be associated with various risks. Training programs along with risk stratification based on the pulmonary hemodynamic response to exercise may enhance the safety of patients with PAH.

Response to exercise in patients with pulmonary arterial hypertension treated with combination therapy.

Pulmonary arterial hypertension (PAH)-specific combination therapy improves pulmonary haemodynamics at rest in patients with PAH; nevertheless, exertional dyspnoea remains. We investigated pulmonary haemodynamic response to exercise, and the relationship to ventilatory efficiency and hypoxaemia in patients with PAH treated with combination therapy. 32 clinically stable patients with PAH undergoing combination therapy underwent cardiopulmonary exercise testing with right heart catheterisation. Haemodynamic impairment was moderate to severe before treatment. However, after treatment it was significantly improved, and the mean pulmonary arterial pressure (mPAP) at rest was <25 mmHg in 13 patients. The mPAP increased significantly from 27.9±10.7 to 45.9±16.7 mmHg (p<0.01) during exercise. The cardiac index increased inadequately, and the total pulmonary resistance (TPR) increased significantly from 5.74±3.42 to 6.58±3.82 Wood units (p<0.01). The mPAP/cardiac output (CO) slope was steep (10.0±6.7 mmHg·min·L-1). It significantly correlated with both the minute ventilation/carbon dioxide output slope (r=0.51, p<0.01) and peripheral arterial oxygen saturation/workload slope (r=-0.41, p=0.02). In addition, the mPAP/CO slope correlated significantly with mPAP at rest (r=0.73, p<0.01) and TPR at rest (r=0.64, p<0.01). Even after pulmonary haemodynamics at rest was significantly improved in PAH patients with PAH-specific combination therapy, the mPAP/CO slope was steep and the steep mPAP/CO slope related to decreased ventilatory efficiency and the severity of hypoxaemia. The mPAP/CO slope was steeper in patients with higher mPAP and TPR at rest.

Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: A systematic review.

BACKGROUND: Balloon pulmonary angioplasty (BPA) has been performed for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or residual pulmonary hypertension after pulmonary endarterectomy (PEA). We performed a systematic review to assess the efficacy and safety of BPA, especially compared to medical treatment or PEA. METHODS: We reviewed all studies investigating pre- and post-treatment pulmonary hemodynamics, mortality, or complications from three electronic databases (PubMed, Cochrane Library, Japan Medical Abstracts Society) prior to February 2017. From 26 studies retrieved, we selected 13 studies (493 patients): the 10 most recent ones including complete data from each institution, one study of residual pulmonary hypertension, and two studies comparing BPA with medical treatment or PEA. RESULTS: No randomized controlled or prospective controlled studies comparing BPA with medical treatment or PEA were reported. The early mortality of BPA ranged from 0% to 14.3%; lung injury occurred in 7.0% to 31.4% (average sessions, 2.5-6.6). Mean pulmonary arterial pressure decreased from 39.4-56 to 20.9-36 mm Hg, and the 6-min walk distance increased from 191-405 to 359-501 m. The 2-year mortality of 80 patients undergoing BPA was significantly lower compared to 68 patients receiving medical treatment (1.3% vs. 13.2%); the risk ratio was 0.14 (95% confidence interval: 0.03-0.76). No significant difference was observed in the 2-year mortality between BPA (n=97) and PEA (n=63) patients. CONCLUSIONS: This systematic review suggests that BPA improves hemodynamics, has acceptable early mortality, and may improve long-term survival compared with medical treatment in inoperable CTEPH patients.