A prediction model of simple echocardiographic variables to screen for potentially correctable shunts in adult patients with pulmonary arterial hypertension associated with atrial septal defects: a cross-sectional study.

During the routine follow-up of adult patients with pulmonary arterial hypertension associated with atrial septal defects (ASD-PAH), the suitability of shunt closure depends on the invasive right heart catheterization (RHC). It is difficult to grasp the timing of RHC shunt closure for moderate-severe PAH. This retrospective cross-sectional study was designed to investigate which echocardiographic variables are related to pulmonary vascular resistance (PVR) in adult ASD-PAH patients and propose a method using echocardiographic variables to screen for patients where shunt closure is suitable. A total of 139 adult ASD-PAH patients with a PASP ≥ 60 mmHg measured by transthoracic echocardiogram (TTE) were included in this study. All RHCs were performed within a week after TTE. The Correctable shunt was defined as PVR ≤ 4.6 wood units (WU). Multivariate regressions were performed with echocardiographic variables. The nomogram of prediction model was constructed by the predictors of PVR ≤ 4.6 WU by multivariate logistic regression analysis. Multivariate linear regression revealed that TAPSE (tricuspid annular plane systolic excursion)/pulmonary artery systolic pressure (PASP) measured by TTE was negatively associated with PVR (ß per SD: - 1.84, 95%CI - 2.62, - 1.06). Multivariate logistic regression showed that TAPSE/PASP and pulmonary valve (PV) peak velocity were positively associated with a potentially correctable shunt (PVR ≤ 4.6 WU) (OR per SD: 2.38, 95%CI 1.34, 4.25, and OR per SD: 2.67, 95%CI 1.26, 5.64, respectively). In receiver operating characteristic analysis, the TAPSE/PASP + PV peak velocity combined model achieved the best performance (AUC: 0.8584, sensitivity: 83.33%, specificity: 72.16%). Internal verification showed stable performance (AUC: 0.8591, sensitivity: 88.10%, specificity: 68.04%). The net benefit of this model was greater than other models when it came to a wide range probability threshold in decision curve analysis. TAPSE/PASP + PV the peak velocity model may have great value in predicting adult ASD-PAH patients with operability potential, which could help clinicians make the treatment decision for follow-up patients.

High plasma adiponectin is associated with increased pulmonary blood flow and reduced right ventricular function in patients with pulmonary hypertension.

BACKGROUND: Adiponectin is a biomarker closely related to heart failure. However, its role in pulmonary hypertension remains unclear. In this study, we investigated the association between adiponectin and hemodynamic abnormalities, right ventricular function in patients with congenital heart disease associated pulmonary hypertension (CHD-PH). METHODS: Patients with CHD-PH were enrolled in this cross-sectional study. Linear regression analysis was performed to assess the association between adiponectin, N-terminal pro-Brain Natriuretic Peptide (NT-proBNP) and different clinical parameters. Results were depicted as beta-estimates(ß) with 95%-confidence intervals (95% CI). In addition, mediation and receiver operating characteristic curve analyses were used to analyze the relationships among adiponectin, NT-proBNP and right ventricular function. RESULTS: A total of 86 CHD-PH patients were included. The overall mean adiponectin concentration was 7.9 ± 5.8 µg/ml. Log adiponectin was positively correlated with pulmonary circulation index (ß = 2.2, 95% CI 0.5, 4.0), log NT-proBNP (ß = 0.22, 95% CI 0.04, 0.41) and inversely with the tricuspid annular plane systolic excursion (TAPSE, ß = -4.7, 95% CI -8.6, - 0.8). The mediation analysis revealed the association between NT-proBNP and TAPSE was fully mediated by adiponectin (total effect c = - 5.4, 95% CI -9.4, - 1.5, p = 0.013; direct effect c' = - 3.7, 95% CI -7.5, 0.1, p = 0.067). Additionally, the efficiency of adiponectin for detecting right ventricular dysfunction was not inferior to NT-proBNP (AUC = 0.84, 95% CI 0.67-1.00 vs AUC = 0.74, 95% CI 0.51-0.97, p = 0.23). CONCLUSIONS: Adiponectin is closely correlated with pulmonary blood flow and right ventricular function and may be a valuable biomarker for disease assessment in patients with pulmonary hypertension.

Novel Strategy for Predicting Conduction Abnormalities During Transcatheter Closure of Perimembranous Ventricular Septal Defect in Adults.

BACKGROUND: In this study we evaluated the feasibility and efficacy of predicting conduction system abnormalities under 3-dimensional (3D) electroanatomic mapping guidance during transcatheter closure of perimembranous ventricular septal defects (pmVSDs) in adults.Methods and Results:The distribution of the His-Purkinje system (HPS) close to the margins of pmVSDs in the left ventricle was identified using 3D electroanatomic mapping and near-field HPS was further confirmed by different pacing protocols. Of the 20 patients in the study, 17 (85%) were successfully treated by transcatheter intervention. The minimum distance between the margins of the pmVSD and near-field HPS, as measured by 3D electroanatomic mapping, ranged from 1.3 to 3.9 mm (mean [± SD] 2.5±0.7 mm). Five patients with a minimum distance <2 mm had a higher risk (3/5; 60%) for adverse arrhythmic events, whereas patients with a distance >2 mm were at a much lower risk (1/15; 6.7%) of procedure-related conduction block (P=0.032). No other adverse events were recorded during the follow-up period (median 30 months). CONCLUSIONS: A minimum distance between the pmVSD and near-field HPS <2 mm was associated with a relatively high risk of closure-related conduction block. 3D electroanatomic mapping may be helpful in guiding decision making for transcatheter closure and reduce the incidence of adverse arrhythmic events.

Prediction value of pulmonary hypertension in newly identified left ventricular dysfunction among adult patients after patent ductus arteriosus closure.

The present study aimed to propose the pulmonary hypertension for predicting left ventricular dysfunction in adults after patent ductus arteriosus closure. A total of 183 patients (age ≥18 years) after patent ductus arteriosus occlusion were retrospectively collected in this study. In brief, pre-, post-procedure and short-term follow-up transthoracic echocardiography were performed. Simpson's method was used to measure the left ventricular ejection fraction (LVEF), and LVEF less than 50% after procedure was utilized as a criterion to identify left ventricular dysfunction. As a result, 36 (19.67%) patients developed newly identified left ventricular dysfunction. The rate of newly identified left ventricular dysfunction was significantly higher in moderate or severe pulmonary hypertension groups compared to the groups of mean pulmonary artery pressure (mPAP) <25 mmHg (P < .001). Logistic regression analysis showed that elevated mPAP ( ≥25 mmHg) was an independent predictive value for newly identified left ventricular dysfunction (OR = 3.584, 95%CI: 1.186-10.832, P = .024) after adjusting confounders. The ROC curve revealed a good discrimination power for predicting newly identified left ventricular dysfunction (AUC = 0.924, 95%CI: 0.885-0.963, P < .001). Taken together, newly identified left ventricular dysfunction after patent ductus arteriosus closure was prevalent in patients with elevated mPAP. The pre-procedure elevated mPAP is an independent risk factor for the prediction of the newly identified left ventricular dysfunction in adult patients undergoing percutaneous patent ductus arteriosus closure. It is feasible to propose a risk model for predicting post-procedure left ventricular dysfunction and a heart function monitoring in pulmonary hypertension patients.

Metabolic Status in Patients with Operable vs. Inoperable Left-to-Right Shunts.

BACKGROUND Previous studies have shown the prognostic value of insulin resistance, hyperuricemia, and dyslipidemia in clinical outcome of pulmonary arterial hypertension. Whether these metabolic derangements are different between operable and inoperable left-to-right shunts is unknown. MATERIAL AND METHODS Our study included 116 patients with left-to-right shunts (76 with atrial septal defect and 40 with ventricular septal defect) with or without pulmonary arterial hypertension. Operability of defect closure were assessed by cardiac catheterization and patients were subdivided into an operable group or an inoperable group. The metabolic status, including prediabetes, hyperuricemia, dyslipidemia, hypertension and obesity, were compared between groups. RESULTS Patients receiving defect correction had a lower HbA1c (B: 5.52±0.49 vs. 5.71±0.41, p=0.042) and uric acid (C: 358±105 vs. 406±126, p=0.029) but a higher HDLC (D: 1.21±0.33 vs. 1.08±0.22, p=0.017) and BMI (A: 20.4±3.9 vs. 18.8±3.1, p=0.023). Patients in the inoperable group had a higher prevalence of prediabetes (58% vs. 41%, p=0.076), hyperuricemia (37.2% vs. 21.9, p=0.106), dyslipidemia (74% vs. 56%, p=0.049) but a lower prevalence of hypertension (13.9% vs. 30.1%, p=0.049) and obesity (4.6% vs. 12.3%, p=0.301). According to logistic regression, only HbA1c (1.76 (0.53, 2.99), HR (95% CI), p=0.005) remained significant for pulmonary vascular resistance. CONCLUSIONS Although prediabetes, hyperuricemia, and dyslipidemia were all more prevalent in patients with inoperable left-to-right shunts, only prediabetes was found to be significantly associated with higher pulmonary vascular resistance.

Riociguat for the treatment of pulmonary hypertension: Chinese subgroup analyses and comparison.

OBJECTIVE: PATENT-1 and CHEST-1 were pivotal, international phase III trials assessing riociguat for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Here we compare Chinese patients from these studies with the overall populations, and report the clinical effect and safety of riociguat in Chinese patients with PAH and CTEPH. METHODS: PATENT-1 was a 12-week, randomised, double-blind, placebo-controlled trial of riociguat (maximum 2.5 mg three times daily or 1.5 mg three times daily (exploratory) in patients with PAH. CHEST-1 was a 16-week, randomised, double-blind, placebo-controlled trial of riociguat (maximum 2.5 mg three times daily) in patients with inoperable CTEPH or persistent/recurrent pulmonary hypertension after pulmonary endarterectomy. The primary endpoint in each study was change from baseline to study end in 6 min walking distance (6MWD). Secondary endpoints included pulmonary vascular resistance (PVR), N-terminal prohormone of brain natriuretic peptide, WHO functional class (FC), and time to clinical worsening. RESULTS: Chinese patients in PATENT-1 (n=77) and CHEST-1 (n=32) were younger and had better baseline 6MWD and WHO FC versus the overall population. Riociguat increased 6MWD versus placebo in Chinese patients in PATENT-1 and CHEST-1, with a greater increase observed in CHEST-1 (least-squares mean differences +46 m and +102 m in PATENT-1 and CHEST-1, respectively). Riociguat also improved several secondary endpoints in both studies, and was well tolerated. CONCLUSIONS: Chinese patients displayed differences in baseline characteristics versus the overall populations in PATENT-1 and CHEST-1. Riociguat improved 6MWD, PVR, WHO FC, and other clinical outcomes in Chinese patients with PAH or CTEPH. TRIAL REGISTRATION NUMBER: PATENT-1: NCT00810693, Results; CHEST-1 NCT00855465, Results.

Predictors of long-term outcomes in patients treated with riociguat for pulmonary arterial hypertension: data from the PATENT-2 open-label, randomised, long-term extension trial.

BACKGROUND: Pulmonary arterial hypertension is a chronic disease associated with poor long-term outcomes. Identifying predictors of long-term outcome in pulmonary arterial hypertension is important to assess disease severity and guide treatment. We investigate associations between efficacy parameters and long-term outcomes in patients with pulmonary arterial hypertension receiving riociguat in the PATENT-2 study. We also present safety and efficacy data from the final data cutoff of PATENT-2, where most patients had received at least 2 years of riociguat treatment. METHODS: Eligible patients from the PATENT-1 study entered the PATENT-2 open-label extension, which will continue until all patients transition to the commercial drug. All patients received riociguat individually adjusted to a maximum dose of 2·5 mg three times a day. The primary endpoint was safety and tolerability, assessed with recording adverse events, serious adverse events, discontinuations, and deaths; exploratory assessments included 6-min walking distance (6MWD), WHO functional class, N-terminal prohormone of brain natriuretic peptide (NT-proBNP)concentrations, Borg dyspnoea score, health-related quality of life (EQ-5D score), survival, and clinical worsening-free survival. Association between efficacy parameters and long-term outcomes was assessed using Kaplan-Meier analyses and a Cox proportional-hazards regression model. PATENT-2 is registered at ClinicalTrials.gov, number NCT00863681. FINDINGS: 396 patients entered PATENT-2, of whom 197 patients were receiving riociguat monotherapy and 199 were receiving riociguat in combination with endothelin receptor antagonists or prostanoids, or both. A significant association was noted between 6MWD, NT-proBNP concentration, and WHO functional class and overall survival at baseline (p=0·0006, 0·0225, and 0·0191, respectively), and at follow-up (p=0·021, 0·0056, and 0·0048, respectively). Riociguat was well tolerated in PATENT-2. Serious adverse events were recorded in 238 (60%) of the total population, and 45 (11%) patients discontinued treatment because of an adverse event. Improvements in 6MWD, WHO functional class, and NT-proBNP concentrations were maintained after 2 years of treatment. INTERPRETATION: These results support the long-term use of riociguat in patients with pulmonary arterial hypertension, and emphasise the prognostic value of 6MWD, WHO functional class, and NT-proBNP concentrations. FUNDING: Bayer Pharma AG.

Use of clinically relevant responder threshold criteria to evaluate the response to treatment in the phase III PATENT-1 study.

BACKGROUND: In PATENT-1, riociguat significantly improved 6-minute walking distance (6MWD) and a range of secondary end-points in patients with pulmonary arterial hypertension (PAH). We investigated whether riociguat increased the proportion of patients achieving clinically relevant responder thresholds compared with placebo during PATENT-1. METHODS: In PATENT-1, a randomized, double-blind study, treatment-naïve patients or patients on background PAH-targeted therapy with symptomatic PAH received 12 weeks of treatment with placebo, riociguat up to 2.5 mg 3 times daily, or riociguat up to 1.5 mg 3 times daily. Increases in 6MWD ≥40 m, 6MWD ≥380 m, cardiac index ≥2.5 liter/min/m(2), mixed venous oxygen saturation ≥65%, World Health Organization functional class I/II, N-terminal pro-brain natriuretic peptide <1,800 pg/ml, and right atrial pressure <8 mm Hg were chosen as threshold criteria of a positive response. RESULTS: Riociguat increased the proportion of treatment-naïve patients and patients on background PAH-targeted therapy with 6MWD ≥380 m at Week 12 (+21% and +15%, respectively), whereas there was a small reduction in 6MWD in placebo-treated patients for both sub-groups. Riociguat also increased the proportion of treatment-naïve patients and patients on background PAH-targeted therapy achieving World Health Organization functional class I/II (+12% and +19%, respectively) and cardiac index ≥2.5 liter/min/m(2) (+30% and +33%, respectively) at Week 12, whereas there was little change in the respective placebo groups. CONCLUSIONS: Compared with placebo, riociguat increased the proportion of treatment-naïve patients and patients on background PAH-targeted therapy who fulfilled criteria defining a positive response to therapy.

Inaccuracy of doppler echocardiographic estimates of pulmonary artery pressures in adult atrial septal defect patients with pulmonary arterial hypertension.

BACKGROUND: While echocardiography has been a pivotal screening test in pulmonary arterial hypertension (PAH), the presence of structural cardiac defects may affect the ability to reliably predict pulmonary artery pressures (PAPs). This study sought to evaluate the accuracy of Doppler echocardiography (DE) for estimating PAPs in adult atrial septal defect (ASD) patients with PAH. METHODS: A prospective study was carried out to compare the echocardiographic assessment of PAP with the same pressures obtained by right heart catheterization (RHC) in adult ASD patients with PAH who underwent simultaneous DE and RHC. Bland-Altman analyses were performed to evaluate the agreement between DE and RHC measurements of PAPs. RESULTS: Two hundred and fifty-seven patients were included in the study. A significant overestimation of the systolic pulmonary arterial pressure (sPAP) and mean pulmonary artery pressure (mPAP) was reported by echocardiography compared with those by catheterization ((81.8 ± 26.9) mmHg vs. (72.9 ± 26.9) mmHg, P < 0.01; (51.9 ± 16.4) mmHg vs. (41.4 ± 17.2) mmHg, P < 0.01, respectively). Twenty-one percent (55/257) of the patients had PAH when estimated by echocardiography whereas showed normal results in the subsequent catheterization test. Using Bland-Altman analytic methods, the bias for the echocardiographic assessment of the sPAP was 9.1 mmHg with 95% limits of agreement ranging from -24.4 to 42.6 mmHg. For mPAP measurement, the bias was 10.5 mmHg with 95% limits of agreement ranging from -12.4 to 33.4 mmHg. On multiple linear regression analysis, age, gender, body surface area, ASDs' diameter, PVR, diastolic blood pressure, and echocardiographic assessment of right atrial pressure (RAP) explained 68.8% of the total variability in the model (r(2) = 0.688, P < 0.01). CONCLUSION: Inaccuracy was frequently reported in Doppler echocardiographic assessment of the PAP in adult ASD patients with PAH and was often associated with age, gender, body surface area, ASDs' diameter, pulmonary vascular resistance, diastolic blood pressure and echocardiographic estimation of RAP.

[Effects of iloprost combined with low dose tadalafil in adult congenital heart disease patients with severe pulmonary arterial hypertension: a single-center, open-label controlled study].

OBJECTIVE: To evaluate the therapy efficacy of iloprost combined with low dose tadalafil in adult congenital heart disease (CHD) patients with severe pulmonary arterial hypertension (PAH). METHODS: Adult CHD patients with severe PAH were included and divided into the sequential combination therapy group [iloprost: 10 µg/inhalation, 6 times per day for 6 months, and then add oral tadalafil (5 mg/d) till 12 months, n = 32] and upfront combination therapy group [iloprost: 10 µg/inhalation, 6 times per day combined with oral tadalafil (5 mg) for 12 months, n = 36]. Data on 6 min walking test (6MWT), Borg dyspnea score, oxygen saturation measurement, WHO classification, and cardiac catheterization were obtained at baseline, 6 and 12 months. RESULTS: Seventy-two patients were enrolled in the study and 68 patients completed the study. Pulmonary vascular resistance (PVR) was significantly reduced in the sequential combination therapy group[ (12.96 ± 6.48 ) Wood U vs. (16.94 ± 8.11) Wood U, P < 0.05] and in the upfront combination therapy group [(12.45 ± 7.32) Wood U vs. (16.73 ± 9.28) Wood U, P < 0.05] while pulmonary blood flow [(6.77 ± 3.17) L/min vs. (5.08 ± 2.36) L/min, P < 0.05; (6.95 ± 3.32) L/min vs. (5.03 ± 2.32) L/min, P < 0.05], the 6 MWD were significantly increased [(458 ± 59) m vs. (427 ± 65) m, P < 0.05; (494 ± 59) m vs. (436 ± 62) m, P < 0.01], the Borg dyspnea score (2.04 ± 0.72 vs. 2.52 ± 0.79, P < 0.05; 1.72 ± 0.73 vs. 2.51 ± 0.77, P < 0.01) was significantly improved in both groups at 6 months compared to baseline levels. In the upfront combination therapy group, venous oxygen saturation [(68.4 ± 9.3)% vs. (62.9 ± 9.5)%, P < 0.05] and systemic oxygen saturation during exercise[ (87.2 ± 9.7)% vs. (83.1 ± 15.6)%, P < 0.05]at 6 months were also significantly improved compared to baseline. At month 12, significantly lowered pulmonary artery pressure, PVR, Rp/Rs and increased pulmonary blood flow and cardiac index were evidenced in both groups compared to baseline. CONCLUSION: Iloprost combined with low dose tadalafil regimen can effectively reduce PVR, increase 6MWD, and improve cardiopulmonary function in adults CHD patients with severe PAH. Compared with the sequential therapy regimen, the upfront combination therapy regimen can more rapidly improve the clinical symptoms of patients.

Transcatheter closure of large patent ductus arteriosus with severe pulmonary arterial hypertension in adults: immediate and two-year follow-up results.

BACKGROUND: Transcatheter closure of patent ductus arteriosus (PDA) is a well established procedure and an accepted treatment modality for small to moderate-sized PDA. This study aimed to evaluate the immediate and follow-up results of transcatheter closure of large PDAs with severe pulmonary arterial hypertension (PAH) in adults. METHODS: After a complete hemodynamic evaluation differentiating from the reversibility of severe PAH, transcatheter closure of PDA was performed. Patients were followed up clinically and echocardiographically at 24 hours, 1 month, 3 months, 6 months, 12 months and 24 months after occlusion. RESULTS: Twenty-nine patients had successful occlusion, pulmonary artery pressure (PAP), left ventricular ejection fraction (LVEF) and fractional shortening (FS) significantly decreased immediately after occlusion ((106 ± 25) mmHg vs. (50 ± 14) mmHg, P < 0.01; (63.7 ± 7.2)% vs. (51.4 ± 10.1)%, P < 0.01 and (36.9 ± 8.2)% vs. (28.9 ± 8.6)%, P < 0.05, respectively). At 1 month after PDA closure, the signs and symptoms improved markedly in all 29 patients, and PDAs were completely closed and remained closed during the follow-up. Eighteen patients having different degrees of dyspnea were treated with angiotensin converting enzyme inhibitor (ACEI) and/or digoxin after occlusion. Nine patients whose pulmonary vascular resistence (PVR) > 6 Wood units accepted targeted PAH therapy. After 1 to 3 months of peroral drug therapy, their exercise tolerance improved from New York Heart Association (NYHA) class III-IV to NYHA class I. During follow-up, no latent arrhythmias were found, the left atrial diameter (LAD), left ventricular end-diastolic diameter (LVEDD), left ventricular end-systolic diameter (LVESD), left ventricular mass index (LVMI) and pulmonary artery systolic pressure (PASP) decreased significantly (P < 0.05), and FS and LVEF recovered compared to the immediate postclosure state. However, FS and LVEF remained low compared to the preclosure state. CONCLUSIONS: Transcatheter closure of large PDA with severe PAH is feasible, effective, and safe in adults. Significant left ventricular systolic changes may occur after closure of large PDA, and left ventricular function usually recovers within a few months.

Transcatheter closure of patent foramen ovale in Chinese patients with paradoxical embolism. -Immediate results and long-term follow-up-.

BACKGROUND: The aim of the present study was to assess immediate and long-term clinical outcome of Chinese patent foramen ovale (PFO) patients with paradoxical embolism who underwent transcatheter PFO closure. METHODS AND RESULTS: One hundred and ninety-two patients underwent transcatheter PFO closure for secondary prevention of thromboembolic events (TE). During the procedure, 7 patients had frequent atrial premature beats or transient atrial tachycardia in implantation and 1 patient had a transitory ST-elevation in leads II, III and aV(F). These complications converted spontaneously after a few minutes. No cases of procedure-related death or TE were observed during hospitalization. Minor adverse events, including chest discomfort (11%), palpitations (25%) and dyspnea (1%) were reported within 1 month of the procedure. These symptoms had disappeared in most patients by 6-month follow-up. One patient had a new occurrence of migraine at 27 months after the implantation. Within a median follow-up of 49 ± 8 months, no residual shunt of the atrial level was identified and correct positioning of the device was confirmed on transthoracic echocardiography in all patients. No death related to any cause or recurrent TE were recorded. CONCLUSIONS: Transcatheter PFO closure is a minimally invasive procedure with a high success rate, low complication rate and an excellent long-term outcome, and appears to be a wise approach for secondary prevention of recurrent embolic events in symptomatic patients.

Experimental study of the Spider™ patent foramen ovale occluder.

AIM: To evaluate the feasibility, safety and efficacy of the Spider™ patent foramen ovale (PFO) occluder for the treatment of PFOs in an animal model. METHOD: The foramen ovale was punctured to establish an animal model of a PFO. Under fluoroscopic guidance, the PFO was occluded with the Spider PFO occluder. The piglets were electively sacrificed at months 1, 2, 3, 6 and 12 after occlusion for macroscopic and microscopic examination. RESULTS: PFOs were successfully created and occluded percutaneously in 20 piglets. All piglets recovered from the procedure in good condition and completed follow-up. Macroscopic examination of all hearts showed complete closure of the PFO. The position of each device was correct. All devices were completely covered and embedded in pale and smooth tissue. There was no evidence of interference with adjacent structures of the heart or valves. The occluder was partially covered by endocardium after 1 month, almost completely covered by fresh endocardium after 3 months, and embedded within thick fibrous tissues and fresh vessels after 6 months. The tissue covering both occluder discs demonstrated some infiltration of inflammatory cells, including patches of lymphocyte aggregation. This inflammatory cell reaction diminished with increasing distance from the device. Furthermore, the number of inflammatory cells decreased gradually during follow-up and eventually, at 6-month follow-up, the inflammatory response was no longer visible. The 12-month histological findings did not differ from the 6-month findings. CONCLUSION: Transcatheter closure of PFO with the Spider PFO occluder is effective and safe in an animal model. These results support further investigation of this device in animals and, if the results are confirmed, in human clinical trials.

Acute left ventricular failure after transcatheter closure of a secundum atrial septal defect in a patient with hypertrophic cardiomyopathy.

We report a case of acute left ventricular failure at one hour after transcatheter closure of a secundum atrial septal defect (ASD) in a 28-year-old man with hypertrophic cardiomyopathy. Afforded noninvasive mechanical ventilation and the administration of intravenous morphine and high doses of furosemide, the patient exhibited improvement of his clinical condition, reduction of pulmonary congestion at chest X-ray, and satisfactory blood gas analyses in twelve hours. Twenty-four hours later, the patient received oral administration of furosemide and metoprolol. After 7 days the patient was discharged in good clinical condition. At follow-up at 12 months, the patient had remained symptomatically improved from NYHA Class III symptoms before the procedure to Class II symptoms. There was no latent arrhythmia at the follow-up examination. Follow-up transthoracic echocardiography estimated an improvement of the left ventricular function. So, transcatheter closure of a secundum ASD in a patient with hypertrophic cardiomyopathy is feasible, and a thorough understanding of the hemodynamic condition of ASD and hypertrophic cardiomyopathy will reduce the complication of ASD closure.

[Immediately effects of inhaled aerosolised iloprost in adult patients with severe pulmonary hypertension secondary to congenital heart diseases].

OBJECTIVE: To investigate the immediately effects of inhaled aerosolized iloprost in adult patients with severe pulmonary arterial hypertension (PAH) secondary to congenital heart diseases (CHD). METHODS: Adult patients with severe PAH secondary to CHD (n = 165) were included in this study. Right heart catheterization was performed, Pulmonary and systemic blood flow, the oxygen consumption VO(2) (ml/min) were calculated using Fick's principle. Pulmonary vascular resistances (PVR) were calculated with standard formulas and indexed to body surface area. Hemodynamic parameters were measured before and after iloprost inhalation (20 µg). RESULTS: Post iloprost inhalation, heart rate, mean aortic pressure, pulmonary systolic pressure to aortic systolic pressure ratio all remained un changed (P > 0.05), while pulmonary artery pressure (PAP) were significantly reduced and Qp significantly increased from (7.2 ± 4.8) L/min to (9.9 ± 7.2) L/min (P < 0.01), PVR was also significantly reduced from (13.4 ± 8.7) Wood units to (9.5 ± 6.6) Wood units (P < 0.01), and left-to-right shunt volume increased from (3.2 ± 4.4) L/min to (5.5 ± 7.0) L/min (P < 0.01) and right-to-left shunt volume decreased from (1.0 ± 1.0) L/min to (0.7 ± 0.7) L/min (P < 0.01). Subgroup analysis showed that adult patients with patent ductus arteriosus and/or ventricular septal defects are more likely to develop severe pulmonary arterial hypertension or Eisenmenger syndrome than patients with atrial septal defects. CONCLUSIONS: Inhaled Aerosolised iloprost use is effective and safe for adult patients with severe pulmonary arterial hypertension secondary to congenital heart diseases.

En face view of atrial septal defect by two-dimensional transthoracic echocardiography: comparison to real-time three-dimensional transesophageal echocardiography.

BACKGROUND: It is generally believed that three-dimensional (3D) echocardiography can provide unique en face views of atrial septal defects (ASDs), whereas conventional two-dimensional (2D) transthoracic echocardiography (TTE) cannot. The purpose of this study was to develop a special 2D TTE-based en face view of ASDs. METHODS: En face views of ASDs on 2D TTE were obtained in 415 consecutive adult patients and compared with the results of real-time 3D transesophageal echocardiography in 25 of these patients. RESULTS: Acceptable en face views of ASDs on 2D TTE were obtained in 80% of patients, in whom secundum ASDs could be adequately imaged in more standard 2D imaging planes. The ability to visualize en face views of ASDs on 2D TTE was inversely related to their sizes (P<.05). The differences in the major and minor dimensions of ASDs between 2D TTE and real-time 3D transesophageal echocardiography were not statistically significant (P>.05). Conventional 2D transthoracic echocardiographic views significantly underestimated ASD size with superior-inferior directional major axis. CONCLUSION: Two-dimensional TTE can provide en face views of ASDs and their spatial orientations with respect to neighboring structures. This method may provide incremental information to 3D echocardiography in patients with ASDs.

[Quantitative assessment of pulmonary blood flow by echocardiography in patients with congenital heart defect].

OBJECTIVE: To explore the possibility and reliability of echocardiography in quantitative evaluation of pulmonary blood flow in patients with congenital heart disease (CHD). METHODS: Sixty-four patients with left to right shunt congenital atrial septal defect (ASD) underwent echocardiographic examinations of the right upper and lower pulmonary vein blood flow spectrum in the four-chamber face, and the right upper pulmonary vein flow velocity time integral (VTIrupv) and right inferior pulmonary venous flow velocity time integral (VTIrlpv) were calculated according to the heart rate. The VTIrupv and VTIrlpv were compared with the pulmonary blood flow (Qp) calculated by Fick method with right heart catheterization. RESULTS: There was a high correlation between the right lung vein flow velocity time integral measured by the catheter of transthoracic echocardiography and Qp. CONCLUSION: The pulmonary venous flow spectrum measured by echocardiography can be informative of the pulmonary blood flow in patients with CHD. Echocardiography may serve as a potential noninvasive technique to evaluate pulmonary blood flow in these patients.

Transcatheter closure of patent foramen ovale with the Spider patent foramen ovale occluder: a prospective, single-center trial.

BACKGROUND: Transcatheter closure of patent foramen ovale (PFO) is a promising alternative to surgical closure or anticoagulation therapy to prevent paradoxical embolic events in patients with PFO. Several different devices have been used for transcatheter PFO closure. The aim of the present study was to evaluate the safety and feasibility for closure of PFO with a new PFO occluder, the Spider PFO occluder. METHODS: The device was implanted in the PFO patients under fluoroscopy and transthoracic echocardiography (TTE) using a 10 French delivery sheath employing a femoral vein approach. Aspirin was administered at 100 mg/d for six months after occlusion. The clinical and echocardiographic follow-up of patients were performed at the 24th hour, 1st month, 3rd month, 6th month, and 12th month after occlusion, and yearly thereafter. RESULTS: The device was implanted successfully in all 55 patients. No major complications occurred during the perioperative period, such as thromboembolism, occluder dislodgement, infection or myocardial infarction. No residual shunt of the atrial level was shown by transesophageal echocardiography, and no latent arrhythmia or cerebral vessel events occurred in any cases during follow-up ((35 +/- 9) months, range 6 - 51 months). CONCLUSION: Transcatheter closure of a PFO with the Spider PFO occluder is a safe and effective therapeutic option for the secondary prevention of presumed paradoxical embolism. However, randomized trials comparing this device with other devices and therapies have to be performed.