Agitated saline with 10% blood increases number and stability of microbubbles in detection right-to-left shunt by contrast-enhanced transcranial Doppler: an in vitro and in vivo observational study.

Background: Agitated saline (AS) with blood has been shown to have good tolerance and increased efficacy when used in contrast-enhanced transcranial Doppler (c-TCD) to detect right-to-left shunt (RLS). However, little is known about the effects of blood volume on c-TCD results. Our study investigated the characterization of AS with different blood volumes in vitro and compared the c-TCD results in vivo. Methods: In vitro, AS without blood, AS with 5% blood (5% BAS), and AS with 10% blood (10% BAS) were prepared based on previous studies and observed under microscopy. The numbers and sizes of the microbubbles from different contrast agents were compared immediately, 5 min, and 10 min post-agitation. In vivo, 74 patients were recruited. c-TCD was repeated 3 times using AS with different blood volumes in each patient. Signal detection times, positive rates, and classifications of RLS were compared among the 3 groups. Results: In vitro, the AS without blood produced 5.4±2.4/field microbubbles after agitation, the 5% BAS produced 30.4±4.2/field, and the 10% BAS produced 43.9±12.7/field. More microbubbles remained in the 10% BAS than the 5% BAS within 10 min (18.5±6.1 vs. 7.1±2.0/field, P<0.001). The size of the microbubbles from the 5% BAS increased from 9.2±8.2 to 22.1±10.6 µm within 10 min post-agitation (P=0.014), while the 10% BAS changed insignificantly. In vivo, the signal detection times of the 5% BAS (1.1±0.7 s) and 10% BAS (1.0±0.8 s) were significantly shorter than the AS without blood (4.0±1.5 s, P<0.0001). The RLS positive rates were 63.5%, 67.6% and 71.6% in AS without blood, 5% BAS and 10% BAS respectively; however, the differences were not statistically significant. The AS without blood reached 12.2% level III RLS, while the 5% BAS reached 25.7%, and the 10% BAS reached 35.1% (P=0.005). Conclusions: The 10% BAS would be suggested in c-TCD as it addressed larger RLS by increasing the number and stability of microbubbles, and it improves the diagnosis of patent foramen ovale (PFO).

[Effect of calorie-enriched formula on postoperative catch-up growth in infants with cyanotic congenital heart disease: a propective randomized controlled study].

OBJECTIVE: To study the effect of calorie-enriched formula on postoperative catch-up growth in infants with cyanotic congenital heart disease (CHD). METHODS: A total of 100 infants with cyanotic CHD who underwent surgical operation from January to December, 2017, were randomly divided into a high-calorie group (receiving calorie-enriched formula after surgery) and a conventional group (receiving standard formula after surgery), with 50 infants in each group. All infants were followed up for 6 months. The observation indices included body height, body weight, prealbumin, and N-terminal pro-brain natriuretic peptide before surgery, at the time of ventilator weaning and extubation after surgery, and at 1, 3, and 6 months after surgery. Height-for-age Z-score (HAZ), weight-for-age Z-score (WAZ), and weight-for-height Z-score (WHZ) were also assessed. Adverse reactions were recorded for both groups. RESULTS: There were 25 cases (50%) and 21 cases (42%) of malnutrition in the high-calorie group and the conventional group respectively before surgery (P > 0.05). The nutritional status of the two groups improved 6 months after surgery (P < 0.05). At 6 months after surgery, compared with the conventional group, the high-calorie group had a lower proportion of infants with malnutrition (18% vs 36%, P < 0.05) and also a lower proportation of infants with a WAZ score of < -2 (P < 0.05). The infants with malnutrion in the high-calorie group had higher HAZ, WAZ, and WHZ than those in the conventional group (P < 0.05). No gastrointestinal intolerance was observed in both groups during hospitalization. CONCLUSIONS: Compared with the standard formula, calorie-enriched formula can better help with postoperative catch-up growth in infants with cyanotic CHD.

Where the congenital heart disease meets the pulmonary arterial hypertension, FLNA matters: a case report and literature review.

BACKGROUND: Pediatric patients with genetic disorders have a higher incidence of pulmonary arterial hypertension (PAH) regardless of their heart defects. Filamin A (FLNA) mutation is recently recognized to be associated with pediatric pulmonary disorders, however, the clinical courses of PAH related to the mutation were reported in limited cases. Here, we presented a case and pooled data for better understanding of the correlation between FLNA mutation and pediatric PAH. CASE PRESENTATION: The patient was a 8-month-old female with repeated episodes of pneumonia. Physical examination revealed cleft lip, cleft palate and developmental retardation. Imaging examination showed a small atrial septal defect (ASD), central pulmonary artery enlargement, left upper lobe of lung atelectasis, and pulmonary infiltration. Genetic test showed she carried a de novo pathogenic variant of FLNA gene (c.5417-1G > A, p.-). Oral medications didn't slow the progression of PAH in the patient, and she died two years later. CONCLUSIONS: FLNA mutation causes rare but progressive PAH in addition to a wide spectrum of congenital heart disease and other comorbidities in pediatric patients. We highly recommend genetic testing for pediatric patients when suspected with PAH. Given the high mortality in this group, lung transplantation may offer a better outcome.

Understanding the current status of patients with pulmonary hypertension during COVID-19 outbreak: a small-scale national survey from China.

Pulmonary hypertension is a chronic disease developing progressively with high mortality. Pulmonary hypertension patients need persistent medical care; however, limited reports focused on them when there was an outbreak of coronavirus disease 2019 in China. This national survey was aimed to evaluate the overall condition of pulmonary hypertension patients during this period. A questionnaire regarding the living condition of pulmonary hypertension patients during coronavirus disease 2019 was designed by pulmonary hypertension diagnostic experts in Wuhan Asia Heart Hospital. Pulmonary hypertension patients and their family members were invited to participate in this survey online. One-hundred twenty pulmonary hypertension patients and 23 family members participated in the survey; 64.8% (n = 87) participants came from Hubei, and others were from 15 other provinces; 98.6% (n = 141) participants were in home quarantine; 65.8% (n = 79) were pulmonary arterial hypertension associated with congenital heart disease; and 76.7% (n = 92) patients proclaimed their heart function was well maintained at class I or II. One (0.8%) patient was confirmed severe acute respiratory syndrome coronavirus 2 infection. Two (1.7%) patients were hospitalized due to heart function worsening. Nearly 70% (n = 100) participants implied shortage in medications during coronavirus disease 2019 outbreak. A total of 24.2% (n = 29) patients indicated that medications were discontinued due to the insufficient supply. Most of the participants stayed optimistic on either coronavirus disease 2019 outbreak or their pulmonary hypertension disease, and 61.7% (n = 74) patients would go to the hospital for follow-up immediately after outbreak. These preliminary data show pulmonary hypertension patients are able to avoid severe disease when they are in home quarantine. Medication supplement is important for pulmonary hypertension patients when their heart function is well maintained. In addition, there might be increasing requirements of medical care for pulmonary hypertension patients after the outbreak.

Correlation between right ventricular-pulmonary artery coupling and the prognosis of patients with pulmonary arterial hypertension.

This study aimed to analyze the correlation between the efficiency coefficient of right ventricular-pulmonary artery coupling (ηvv) and the prognosis of patients with pulmonary arterial hypertension (PAH).A total of 64 patients who underwent right heart catheterization (RHC) were enrolled and divided into PAH and control groups depending on the RHC results. Pressure and volumetric methods were adopted to analyze the results of RHC and cardiac magnetic resonance imaging examination. The ηvv of patients in 2 groups were calculated, and the relationship between ηvv calculated by the 2 methods and the 2-year prognosis of patients with PAH was evaluated.The hemodynamic index and right ventricular-pulmonary artery coupling parameter of patients with PAH were significantly higher than those in the control group (P < .05). The right ventricular volume parameter in the PAH group was significantly different from that in the control group (P < .05). For patients with PAH, the end-systolic elastance/effective arterial elastance (Ees/Ea) calculated by the volumetric method was significantly related to the prognosis of patients (odds ratio = 0.192, 95% confidence interval: 0.042-0.868, P = .032). When Ees/Ea <0.67 was calculated by the volumetric method, the adverse prognosis of patients with PAH increased significantly (P < .05).The Ees/Ea calculated by the volumetric method may be better an independent factor for the prognosis of patients with PAH.

Guideline implementation and early risk assessment in pulmonary arterial hypertension associated with congenital heart disease: A retrospective cohort study.

INTRODUCTION: Current guidelines emphasize that accurate risk stratification is important for patients with pulmonary arterial hypertension (PAH), however, few suggestions have been specified for PAH associated with congenital heart disease (PAH-CHD). OBJECTIVES: The aim of this study was to propose an accurate and simple system based on current guidelines for risk stratification in PAH-CHD patients during 12-month follow-up. METHODS: We reviewed 288 Chinese PAH-CHD patients between January 2014 and December 2016 in this retrospective cohort study. The low-risk criteria according to 2015 European Society of Cardiology guidelines and the adverse events (AEs) during follow-up were collected. The association between low-risk criteria and AEs was assessed with Cox regression, and a simplified risk stratification system was proposed. RESULTS: There were 105 PAH-CHD patients included in the final analysis. Twenty-nine patients had AEs defined as death, initiation of new or combined medication treatment, or re-hospitalisation because of the PAH worsening. Among the low-risk criteria, WHO/NYHA functional class, 6-minute walking distance (6MWD), NT-proBNP and SvO2 were significantly different between AE and AE-free groups. However, 6MWD (HR = 0.08, 95% CI: 0.03-0.19, P < 0.001) and NT-proBNP (HR = 0.35, 95% CI: 0.16-0.78, P = 0.01) were the only independent predictors of AEs in multivariable model. When taking them into a simplified system for risk stratification, the number of low-risk criteria at diagnosis discriminated the risk of AEs (P < 0.001). CONCLUSIONS: Among the low-risk criteria proposed by current guidelines, 6MWD and NT-proBNP predicted AEs independently for PAH-CHD patients. Simplified risk stratification system by taking these two parameters numerically provides accurate prognostic information in PAH-CHD patients.