Case report of pulmonary hypertension caused by filamin A mutation / 中华实用儿科临床杂志

The clinical data of a case of pulmonary arterial hypertension (PAH) caused by mutation of filamin A( FLNA) in the congenital heart disease center of Wuhan Asian Heart Hospital in September 2017 were subject to retrospective analysis.A 2-year-old girl had clinical characteristics of special face, backward growth and development, repeated pulmonary infections, repeated heart failure, congenital heart disease, and PAH.Genetic detection: FLNA mutation (exon34: c.5417-1G> A), which was a new pathogenic mutation.There were few reports of PAH caused by FLNA mutations.In this study, a new case of pulmonary hypertension caused by FLNA c. 5417-1G>A compound heterozygous shearing mutations was found, which enriched the FLNA mutation spectrum and prompted genetic detection was an important approach to detect the cause of PAH.

Application of ballon atrial septostomy for left heart venting during extracorporeal membrane oxygenation / 中华胸心血管外科杂志

Objective:To discuss whether balloon atrial septostomy(BAS) can provide safe and effective left ventricular venting for venoarterial extracorporeal membrane oxygenation(V-A ECMO).Methods:From March 2017 to January 2019, 9 patients received BAS for left ventricular venting during V-A ECMO treatment in our hospital, including 5 males and 4 females, aged 12-72 years. There were 3 cases of severe myocarditis, 4 cases of low cardiac output after cardiac surgery, and 2 cases of acute myocardial infarction. Basic data, procedure data, outcome and follow-up were recorded.Results:BAS were successfully performed in 9 patients. Procedure time on average was 42.2 min. Anterior mediastinal hematoma occurred in 1 case. There were no other procedure-related complications in the rest cases. No pulmonary edema or thrombosis occurred in all 9 cases during ECMO. No closure procedure was performed.Conclusion:BAS is a safe and effective method for left ventricular venting. The procedure is conductive to the recovery of patients with severe left heart failure.

The evaluation by pulmonary wedge angiography of children with pulmonary artery hypertension ;associated with congenital heart disease / 中国介入心脏病学杂志

Objective To study pulmonary wedge angiography ( PWA ) with hemodynamic the evaluation of children with congenital heart disease and pulmonary artery hypertension ( PAH) . Methods Hemodynamic measurement and pulmonary wedge angiography were performed in 50 children with congenital heart disease. Comparison and analysis were made from the data obtained from PWA and catheterization. Results After PWA, the patients were categorized into 3 groups according to the measured hemodynamics parameters:group A [ n=15, patients with normal mean pulmonary artery pressure ( mPAP≤25 mmHg) and normal pulmonary vessel resistance (PVR﹤300 dyne?s?cm5)], group B [n=24, patients with PAH (mPAP﹥25 mmHg) but normal PVR] and group C (n=11, patients with PAH and elevated PVR (PVR≥300 dyne?s?cm5). Rote of tapering (ROT) was significant lower in group C than in group A and B (F=42. 559,P﹤0. 05). Pulmonary circulation time (PCT) was higher in group C than in group A and B (F=6. 037,P﹤0. 05). ROT correlated negatively with PVR (r = -0. 606, P ﹤0. 05). PCT index correlated positively with PVR (r=0. 783,P=0. 01). There was no significant correlation between PCT and mean pulmonary artery hypertension (mPAP). Conclusions PWA may help to make quantitative analysis of the pulmonary vascular status in patients with congenital heart disease.

Therapeutic effect and safety of ambrisentan on Eisenmenger syndrome / 心血管康复医学杂志

Objective:To observe therapeutic effect of ambrisentan on Eisenmenger syndrome (ES) .Methods:A total of 79 ES patients ,who hospitalized in Wuhan Asia Heart Hospital from Apr 2011 to Mar 2014 ,were divided into ambrisentan group (n=38 ,received digoxin combined ambrisentan treatment) and routine treatment group (n=41 , received digoxin treatment) ,and both groups were treated for 12 weeks .The 6min walking distance (6MWD) , WHO functional class (WHO FC) of pulmonary hypertension etc .were observed in both groups before and 12-week after treatment .Changes of hemodynamic parameters ,including mean pulmonary artery pressure (mPAP) ,total pulmonary resistance (TPR) and ratio of pulmonary circulating flow to system circulating flow (Qp/Qs) etc .,were observed in ambrisentan group before and after treatment . Results:Compared with before treatment , after 12 weeks ,6MWD significantly rose ,TPR significantly reduced ,Qp/Qs significantly increased and WHO FC signifi-cantly improved in ambrisentan group ,P<0.05 or <0.01. Compared with routine treatment group after 12 weeks , there was significant rise in 6MWD [ (356.8 ± 74.1) m vs .(393.7 ± 69.2) m] ,significant reduction in BNP level [(832.5 ± 422.2) ng/L vs .(646.6 ± 297.1) ng/L] and significant improvement in WHO FC (class I/II ,67.5% vs . 86.8% ) in ambrisentan group ,P<0.05 or <0.01. No patient died ,the adverse reactions were mild in ambrisentan group ;one case died in routine treatment group .Conclusion:Oral ambrisentan can significantly improve heart func-tion and exercise capacity in patients with Eisenmenger syndrome ,and it possesses good safety and tolerance .

Feasibility of simultaneous cardiac catheterization and 2D echocardiogram in the measurement of right rentricular pressure-volume loops / 中国介入心脏病学杂志

Objective To investigate the feasibility of the simultaneous measurement of right ventricular pressure-volume loops by cardiac catheterization and 2D electrocardiogram. Methods Patients referred for pulmonary hypertension underwent right heart catheterization in our hospital between June 1st, 2015 and June 1st, 2017 are to be enrolled in this study. The right ventricular volume was measured simultaneously by catheter and electrocardiogram. The pressure-volume loops were constructed by the parameters of the pressure and volume in the same cardiac cycle. Results The study completed in four cases and their pressure-volume loops were drawn. The obtained images were irregular and there was no relationship among them. As a result, the construction was a failure. Conclusions The construction of the right ventricular pressure-volume loops of pulmonary hypertension patients by simultaneous catheterization and 2D electrocardiogram is difficult to overcome the technology defects.

TOF treatment for early postoperative use of endothelin receptor antagonist for the BNP impact study / 中华胸心血管外科杂志

Objective To determine the brain natriuretic peptide(BNP) levels and the effects of endothelin receptor antagonist(ERA) on BNP levels in patients with tetralogy of Fallot(TOF) recently surgical repaired.Methods During January 2010 to January 2012,32 cases of TOF after surgical repaired in hospital were selected.There are 20 males and 12 females,Aged 4 years to 18 years [mean age (7.64 ± 3.75) years] in age.All patients underwent enhanced CT to evaluate the pulmonary vessels and left ventricular before surgery arrangements.As the surgeries done,the patients were grouped randomly as either A or B.All 14 patients in group A started to follow the recommended dosage of bosentan within 3 days after surgery.Meanwhile,all 18 in group B had not taken bosentan or any other ERAs since the surgeries.Both group was evaluated and examined with echocardiography and blood test at the 10th day after surgery.Results None of the patients died within 10 days after surgery.BNP levels of group A was significantly lower than of group B.Inotropic score of group A was markedly lower,too.However,although group A showed mildly advantages in tricuspid regurgitation,pulmonary regurgitation,ratio of RV/LV end-systolic dimension and liver functions,there was no statistically significant difference.Conclusion For patients with tetralogy of Fallot,early use of ERAs after surgical repaired could reduce the use of inotropic agents and significantly decrease the BNP levels when discharged.

Feasibility study of interventional treatment for membranous ventricular septal defect in infants less than three years of age / 介入放射学杂志

Objective To study the feasibility and safety of interventional closure for the treatment of infant’s perimembranous ventricle septal defect (PmVSD). Methods During the period from Jan. 2012 to Jan. 2013, a total of 229 single PmVSD infants less than three years of age were admitted to authors ’ hospital. The infantile patients were divided into intervention group (n = 88) and surgery group (n = 141). Patients in the intervention group underwent interventional closure procedure , while patients in the surgery group received conventional cardiac surgery. The operation success rate , the main and minor complication rates, the operation time, the postoperative hospitalization days, the incidence of postoperative pulmonary infection and the medical cost were recorded , and the results were compared between the two groups. Results No statistically significant differences in the operation success rate, the main complication rate, the postoperative hospitalization days and the medical cost existed between the two groups (P > 0.05). But the minor complication rate and the operation time of the intervention group were better than those of the surgery group. In the surgery group, the minor complication was mainly the respiratory infection, which was manifested as higher leukocyte count, higher C-reactive protein level, higher myocardial damage marker level as well as higher vasoactive drug scores in 24 hours after the operation , and the above items were significantly higher than those in the intervention group. Conclusion For the treatment of infant’s perimembranous ventricle septal defect, percutaneous transcatheter closure is clinically feasible. This technique is safe and reliable with obvious advantages when the indication is strictly observed and the procedure is carefully manipulated. This treatment can partly replace the conventional surgery.

Can PDA patient in early stage of Eisenmenger syndrome accept interventional surgery ? / 中国介入心脏病学杂志

Objective To explore whether the PDA patients in the early stage of Eisenmenger syndrome can accept interventional surgery. Methods Three patients were choose from the “National Multicenter Prospective Registration Study on Pulmonary Artery Hypertension”, Clinical assessment, as well as examinations and registrations associated with PAH, was conducted in accordance with relevant provisions of the registration study within 1 wk prior to surgery. The way of the interventional treatment were right heart catheterization and pulmonary vasodilator testing (a capsule of iloprost solution for inhalation), and measurement of the pulmonary arterial pressure, descending aortic pressure, ratio of pulmonary to systemic blood flow, ratio of pulmonary to systemic blood pressure, pulmonary capillary wedge pressure [mean≤15 mmHg(1 mmHg=0.133 kPa)], before and after testing. After clear the results of pulmonary vasodilator test all patients underwent transcatheter closure testing, if it was positive,the amplatzer was released if her family members’ approved;Conversely, the amplatzer was quickly withdrawn with negative results, and symptomatic treatment was conducted based on the patient’s clinical symptoms. Results The first cases pulmonary vasodilator test was negative, but positive in transcatheter closure testing. Satisfaction immediate effect of surgery, and the occluder was released with her family members’ approval. patients of Example 2 and Example 3 were negative in pulmonary vasodilator testing and transcatheter closure testing, can not released the occluder. Conclusions Some Patents Ductus Arteriosus (PDA) patients in early stage of Eisenmenger syndrome is feasible to accept interventional therapy , try plugging test is may be an important indicator of prognostic assessment.

Clinical analysis of percutaneous balloon pulmonary valvuloplasty in the treatment of 52 patients with pulmonary stenosis / 中国介入心脏病学杂志

Objective To investigate the safety and effectiveness of percutaneous balloon pulmonary valvuloplasty (PBPV) in the treatment of pulmonary stenosis (PS). Methods The subjects were 52 patients with PS from 2007 to 2012, including 24 male and 28 female. Their age ranged from 1 year old to 56 year old and mean age was 9.2±7.6 years old. Their body weight ranged from 10 kg to 60 kg and mean weight was 17.3±8.9 kg. All patients were assessed with electrocardiogram (ECG), chest X-ray, right heart catheterization and right ventricular angiagraphy before the procedure. Results All 52 patients received PBPV successfully. Comparing the transvalvular pressure gradient before and after the operation, it decreased from 86.3±27.6 mmHg (1 mmHg=0.133 kPa) to13.8±7.7 mmHg, and statistically difference (P<0.01). Among these patients, one patient occurred ventricular fibrillation and Aspen syndrome (hypoxie-ischemic encephalopathy), one patient occurred respiratory and cardiac arrest, 24 patients occurred transient arrhythmia, one patient had hypotension, one patient had bradycardia and one patient showed desaturation. All these symptoms resolved after appropriate treatment during the PBPV. Two patients had hematoma in the puncture point and 2 patients had reactive infundibular stenosis after PBPV. Conclusions Performing the percutaneous balloon pulmonary valvoplasty on the patients with pulmonary stenosis is safe and effective. PBPV causes less pain and can be used as the preferred treatment for pulmonary stenosis.

Clinical observation of the effect of one stop hybrid approach for tetralogy of fallot with aorta pulmonary collateral arteries / 中国介入心脏病学杂志

Objective To summarize the clinical effects of one stop hybrid approach for treating the tetralogy of fallot complicated with aorta pulmonary collateral arteries. Methods From Janu 2008 to June 2013, 79 cases of tetralogy of fallot were complicated with aorta pulmonary collateral arteries with mean age (5.4±3.9) years and weight (18.2±5.7) kg. All patients were diagnosed by echocardiography examination and accepted 64-row CT for demonstration of no dysplasia in pulmonary ressels, McGoon index was (1.7±0.6) and Nakata index was (176.7±7.3). 76 cases had aorta pulmonary collateral arteries for preliminary screening, and accepted cardiovascular angiography before operation. 3 cases were misdiagnosed before surgery and were confirmed by cardiovascular angiography after operation. Results There were 4 deceased cases with an operative mortality of 5.1%. 1 case died of lung infection and 3 cases died of refractory heart failure. In all aorta pulmonary collateral arteries, the smallest diameter was 2.5 mm, the largest was 9.4 mm, average (5.3±2.1) mm. 2 to 21 coils were implanted in respective case with an average of 10.2 coils per case. The maximum number of coils implanted in 1 single ressel was 9. The average number of ressels occluded was 3.7 (range from 1 to 11 ressels) in each case. One case received second closure due to re-open of collateral arteries on neo-vasculanigation. 14 cases had lung infection and 3 cases had pulmonary edema post operation. 75 cases were followed up for 3-72 months. 4 cases with post operative heart function of NYHA gradeⅢtoⅣand all other cases with gradeⅡon higher. Conclusions Peri-operative hybrid management for aorta-pulmonary collateral arteries can increase the operative success rates and reduce complications.

Clinical characteristics of 195 Chinese patients with WHO Class I pulmonary hypertension / 中华心血管病杂志

<p><b>OBJECTIVE</b>To analyze the clinical characteristics of WHO Class I pulmonary hypertension (PAH) in central China.</p><p><b>METHODS</b>Data was collected as a part of prospective registry of PAH through Jan. 2009 to Oct. 2013 in Wuhan Asia Heart Hospital. A total of 195 patients were recruited including 144 cases with congenital heart disease with pulmonary hypertension (CHD-PAH) and 51 cases with idiopathic pulmonary hypertension (IPAH).</p><p><b>RESULTS</b>The age of all patients ranged from 1 to 68 years (mean (27.5 ± 13.2) years), 129 cases were female (66.2%). WHO Class I PAH accounted for 91.1%, CHD-PAH 67.3%, IPAH 23.8%, and other 8.9%.WHO function class III/IV in newly diagnosed PAH accounted for 32.3%, the mean 6MWD was (397 ± 74) m. For patients with IPAH, the median time period between onset of symptoms and diagnosis by right heart catheterization was 38 months. The mean pulmonary pressure, pulmonary vascular resistance index, cardiac index of patients with IPAH and CHD-PAH were measured by the right heart catheterization and there was no difference between the two groups. Acute pulmonary vasodilator testing was negative in all patients in this cohort. Cardiac function was improved in the 121 cases who received the targeted drug treatment and 1 patient died out of these 121 patients while 5 cases died out of patients receiving conventional therapy.</p><p><b>CONCLUSION</b>In Central China, Class I pulmonary hypertension is the most predominant type of PAH, the cardiac function and hemodynamic indexes of these patients were significantly impaired at the time of first PAH diagnosis. Most of the patients accepted targeted drug treatment of pulmonary hypertension, but the drug dose used for the targeted drug treatment is not effective enough in these patients.</p>

Aorta-pulmonary septal defect and aortic origin of the right pulmonary artery with interruption of the aortic arch: a clinical analysis of 5 cases / 中华胸心血管外科杂志

Objective Reviewing the experience in the diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of the aortic arch. Methods Reviewed clinical data from medical records for5 patients (4 boys and 1 girl, age from 1 to 18 years on admission) who had been treated in Wuhan Asia Heart Hospital between 2005 and 2009. Results All cases had type A interruption of the aortic arch ( according to Celoria and Patron's Classification) , type Ⅱ aorta-pulmonary septal defect( according to Mori Classification) and aortic origin of right pulmonary artery complicated with interruption of the aortic arch. Large patent ductus arteries were observed in all cases,and no cardiac abnormality such as ventricular septal defect was found. All patients received echocardiography and spiral CT examination, and 3 of them over 10 years of age received cardiac catheterization and angiography for the evaluation of the pulmonary artery pressure. Two patients, at the age of 14 and 18 years, had severe pulmonary hypertension and declined to receive the operation. One of the 2 cases had hemoptysis and was given prostacyclin ( Iloprost solution for inhalation, a drug for pulmonary artery hypertention ) for reducing the pulmonary pressure. The patient now has an improved cardiac fu0ction without recurrent hemoptysis. One 14-year-old case gave up the operation because of the financial problem and failed to communicate with us after discharge. Radical surgery was performed in the remaining three cases, one had serious infection in the lung and died 11 days after the operation, in one case ( 2 years old) pulmonary artery hypertension has been persisted even though drug therapy was given for a long time and was found at a poor cardiac function 18 months after the operation. One patient recovered well under routine drug therapy and has been followed-up. Conclusion Aorta-pulmonary septal defect and aortic origin of right pulmonary artery with interruption of the aortic arch ( with intact ventricular septum) are different from the classic triad of inter ruption of the aortic arch, consisted of the interruption of the aortic arch, patent ductts arteriosus and ventricular septal defect.It should be considered as a special kind of cardiovascular malformation syndrome. Echocardiography can be used for the preliminary diagnosis of aorta-pulmonary septal defect and aortic origin of right pulmonary artery with interruption of the aortic arch. The final diagnosis and decision for surgery should be based on the combined information from cardiac catheterization,cardiovascular angiography and spiral CT examination. Pulmonary artery pressure and resistance are the most important factors,because they have great effects on the duration of surgery and the outcome. A radical operation is necessary for all cases, but the mortality rate is high. The prognosis would be great if only the patient can survive successfully during the peri-operative period, a period with a poor cardiac function, potential lung infection and the pulmonary artery hypertension.