“Treat-Repair-Treat”:Management of Left Main Coronary Compression by a Pulmonary Artery Aneurysm in a Patient with Atrial Septal Defect and Significant Pulmonary Hypertension

Left main coronary compression syndrome(LMCS)may complicate pulmonary artery aneurysms(PAA),usually developed in the context of pulmonary arterial hypertension(PAH).We report the case of a 51-year-old female patient with an atrial septal defect(unsuitable for device closure)complicated by a PAA generating a 90%left main stenosis.The significant PAH held us back from immediate surgery.After specific dual PAH-targeted therapy(sildenafil and bosentan),the atrial septal defect could be closed with a unidirectional valved patch;the PAAinduced LMCS was treated by reductive arterioplasty.The postoperative course was uneventful.Follow-up showed clinical improvement,but PAH treatment was still needed.After three months,coronary angiography showed only an insignificant residual left main stenosis,proving that reductive pulmonary arterioplasty was effective in treating LMCS.Any PAA requires further evaluation for LMCS,a dangerous but treatable complication.The“treat-repair-treat”strategy and shunt-closure with a unidirectional valved patch can both improve surgical prospects of LMCS with shunt-related PAH.

Anatomy in Patients with 22q11 Deletion and Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals

We performed a retrospective analysis of patients with and without 22q11 deletion undergoing surgery for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals between January 2004 and August 2009 at our institutions. Information was collected on collateral origin, arch sidedness, presence of central pulmonary arteries, and presence of an aberrant subclavian vessel. While patients with 22q11 deletion were more likely to have collateral origin from brachiocephalic vessels, patients without 22q11 deletion were more likely to have collateral origin from the descending aorta. There was no significant difference in arch sidedness or the presence of central pulmonary arteries. Patients with 22q11 deletion were more likely to have an aberrant subclavian artery (15/46 vs 5/54, p < 0.05), whether a left or right arch was present. Nine of the fifteen 22q11 deletion patients had a collateral originating from an aberrant subclavian artery. In time, genomic and embryologic research may help determine the exact mechanisms by which 22q11 deletion contributes to the development of congenital heart disease such as pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals.

Discharge planning for children with ventricular septal defect and pulmonary arterial hypertension in China

Objective:To evaluate the effectiveness of discharge planning on maternal caring knowledge,maternal caring behavior,maternal discharge readiness and the rehospitalization of children with ventricular septal defect and pulmonary arterial hypertension(VSD-PAH).Background:Children with congenital heart disease(CHD)with pulmonary arterial hypertension(PAH)have more complications after surgery than those without PAH.Discharge planning is an effective strategy to help children leave the hospital safely,and receive appropriate care after discharge.Methods:A quasi-experimental design was used.Sixty children and their mothers were recruited and divided into two groups:the control group received conventional care,the intervention group received both conventional care and additional discharge planning care.Results:(1)After admission,maternal caring knowledge between the two groups was similar.(2)At discharge,maternal discharge readiness,maternal caring knowledge and maternal caring behavior in the intervention group was significantly higher compared to the control group(t=3.35,p=0.001;F=84.74,p<0.001;F=23.82,p<0.001).This difference persisted after discharge,and was evident at one month and three months after discharge.(3)However,no significant difference in the readmission rate of children after discharge was evident between the two groups.Conclusions:Discharge planning improves the maternal discharge readiness,maternal caring knowledge and maternal caring behaviors.However,this planning did not reduce the readmission rate of children with CHD-PAH.

Evaluation of Biventricular Volume and Systolic Function in Children with Ventricular Septal Defect and Moderate to Severe Pulmonary Hypertension Using Real-Time Three-Dimensional Echocardiography

Background: Real-time three-dimensional echocardiography (RT-3DE) could obtain ventricular volume andejection fraction rapidly and non-invasively without relying on ventricular morphology. This study aims to useRT-3DE to evaluate the changes in biventricular volume and systolic function in children with ventricular septaldefect (VSD) and moderate to severe pulmonary hypertension (PH) before surgery. Methods: In this study18 children with VSD and moderate to severe PH (VSD + PH Group) and 18 healthy children of the sameage (Control Group) were recruited. Biventricular volume and systolic function were evaluated by RT-3DE.The measurements included: left and right ventricular volume indexed to body surface area (BSA), stroke volume(SV) indexed to BSA, and ejection fraction (EF). Results: The results showed left and right ventricular volumeindexed to BSA and SV indexed to BSA were significantly increased in VSD + PH Group (VSD + PH Groupvs. Control Group), LVEDV/BSA (ml/m2): 48.67 ± 21.46 vs. 25.59 ± 6.96, RVEDV/BSA (ml/m2): 55.98 ±15.35 vs. 27.69 ± 4.37, LVSV/BSA (ml/m2): 24.08 ± 9.30 vs. 15.14 ± 4.29, RVSV/BSA (ml/m2): 26.02 ± 8.87 vs.14.11 ± 2.89, (P < 0.05). While for EF in VSD + PH Group decreased (VSD + PH Group vs. Control Group),LVEF: 50.93 ± 7.50% vs. 59.38 ± 7.24%, RVEF: 45.84 ± 7.71% vs. 51.05 ± 6.90% (P < 0.05). Conclusion: Inchildren with VSD and moderate to severe PH, increased biventricular volume and decreased systolic functionwere observed with RT-3DE, but biventricular systolic function remained within acceptable limits. The childrenin this study recovered well after surgery without serious perioperative complications, suggesting that biventricularsystolic function may help facilitate the surgical decision-making process in children with VSD and moderate-toseverePH.

MORPHOMETRIC ANALYSIS OF LUNG BIOPSY TISSUE IN PATIENTS WITH TETRALOGY OF FALLOT AND VENTRICULAR SEPTAL DEFECT WITH PULMONARY HYPERTENSION

The pulmonary vasculature from 24 children with Tetralapy of Fallot (TOF)and ventricular septal defect with pulmonary hypertension (VSD/PH) war studied morphometrically. The study showed hypoplasis of small arteries in TOF There was a decrease in pulmonary artery size, but in the intra-acinar region small arteries were increased in number with mild intimal change. The pulmonary vascular resistance was normal postoperatively. The pulmonary vasculature in VSD/PH displayed a variable degree of obstructive lesions. The pulmonary arterial pressure remained elevated postoperatively in patients with severe pulmonary obstructive diseases.

Left Pulmonary Artery Sling Associated with Patent Ductus Arteriosus and Atrial Septal Defect: Evaluation with Multidetector CT

We report a case of left pulmonary artery sling associated with patent ductus arteriosus and atrial septal defect in a 21-month-old child. 256-slice MDCT provides valuable information, such as abnormal origin of the left pulmonary artery, the relationship between pulmonary artery and airway, the diameter of the patent ductus artery and atrial septal defect. The information is helpful in diagnosis, pre-operative evaluation and post-operative follow-up of LPS.

Aorta-pulmonary septal defect and aortic origin of the right pulmonary artery with interruption of the aortic arch:a clinical analysis of 5 cases

Objective Reviewing the experience in diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of aortic arch. Methods Clinical data from medical records for 5 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

Evaluation of Left Ventricular Rotation and Twist Using Speckle Tracking Imaging in Patients with Atrial Septal Defect

Speckle tracking imaging （STI） was employed to investigate the effect of right ventricular （RV） volume and pressure overload on left ventricular （LV） rotation and twist in 35 patients with atrial septal defect （ASD）, 18 of which with pulmonary hypertension, and 21 healthy subjects serving as controls. The peak rotations of 6 segments at the basal and apical short-axises and the average peak rotation and interval time of the 6 segments in the opposite direction during early systolic phase were measured respectively. LV twist versus time profile was drawn and the peak twist and time to peak twist were calculated. LV ejection fraction （EF） was measured by Biplane Simpson. Compared to ASD patients without pulmonary hypertension and healthy subjects, the peak rotations of posterior, inferior and postsept walls at the basal level were lower （P〈0.05）, and the average counterclockwise peak rotation of 6 segments at the basal level during early systolic phase was higher （P〈0.05）, and the average interval time was delayed （P〈0.05）. LV peak twist was also lower （P〈0.05）, and had a significant negative correlation with pulmonary arterial systolic pressure （r=-0.57, P=0.001）. No significant differences were found in LVEF among the three groups. It was suggested that although RV volume overload due to ASD has no significant effects on LV rotation and twist, LV peak twist is lower in ASD patients with pulmonary hypertension. Thus LV twist may serve as a new indicator of the presence of pulmonary hypertension in ASD patients.

THE EXPESSION OF ENDOTHELIN-1 IN VENTRICULAR SPETAL DEFECT WITH PULMONARY HYPERTENSION

Objective To research the expression of endothelin-1 (ET-1) in vascular of lung, endocardium and myocardial vascular of congenital heart disease (CHD) ventricular septal defect (VSD) with pulmonary hypertension (PH). Methods The Streptavidin-peroxidase (SP) immunoassay was used to measure the expression of ET-1 in pulmonary arteriola, pulmonary veinlet, endocardium and endangium of vasa coronary of 20 cases VSD of CHD with PH, and contrast the expression level of these 20 cases VSD of CHD with PH. Results The expression of ET-1 PH patients in pulmonary arteriola, endocardium, and endangium of vasa coronary was much higher than that of the control group (P<0.05), but there was no significance variance in expression level of pulmonary veinlet between two groups (P>0.05). Conclusion If VSD of CHD was accompany with PH, the degree of PH has a positive correlation with the amount of ET-1 in pulmonary arteriola, ET-1 may be the cause of dynamic PH, and also the acceleration factor of the PH. However, the amount of ET-1 in endocardium and endangium of vasa coronary, may have significant connection with the myocardium hypertrophy in dynamic PH.

Home-made fenestrated amplatzer occluder for atrial septal defect and pulmonary arterial hypertension

We report the management of a patient with secundum atrial septal defect （ASD） and severe pulmonary hypertension. A 65-year-old male with recently diagnosed atrial septal defect was referred to our centre for decompensated right heart failure with rest and exercise induced dispnea and severe pulmonary hypertension. Right heart catheterization confirmed a mean pulmonary pressure of about 55 mmHg and a Qp/Qs of 2.7. An occlusion test with a compliant large balloon demonstrated partial fall of pulmonary arterial pressure. The implantation of a home-made fenestrated Amplatzer ASD Occluder （ASO） was planned in order to decrease left-to-right shunt and promote further decrease of pulmonary arterial pressure in the long-term. Thus, by means of mechanical intracardiac echocardiography study with a 9F 9 MHz Ultralce catheter （Boston Scientific Corp.）, we selected a 34 mm ASO for implantation. Four millimeter fenestration was made inflating a 4 mm non-compliant coronary balloon throughout the waist of the ASO, which was successfully implanted under intmcardiac echocardiography. After six months, a decrease of pulmonary arterial pressure to 24 mmHg and full compensated right heart failure was observed on transthoracic echocardiography and clinical examination. This case suggests that Wanscatheter closure with home-made fenestrated ASD in elderly patients with severe pulmonary hypertension is feasible.

Our Experience in 33 Patients of Multiple Ventricular Septal Defect Closure

Study: Retrospective study of 33 patients with multiple VSD during March 2009 to March 2014. Technique: The defects were located by injecting the cardioplegia solution into left atrium after occluding the pulmonary artery. Through right atrial approach, the large ventricular septal defects were closed by pericardial patch. The smaller ventricular septal defects were closed directly. Results: Out of 33 patients, 20 patients were multiple ventricular septal defects, 5 patients were atrioventricular canal defect with multiple ventricular septal defects and 8 patients were transposition of great arteries with multiple ventricular septal defects. All the patients had severe pulmonary arterial hypertension. The mean age and body weight at repair was 4.51 months and 5.41 kg respectively. Failure to locate additional ventricular septal defects happened in 1 patient where the pulmonary artery banding was done. Six patients had residual ventricular septal defect after surgery, and no significant left to right shunted. One patient had permanent pacemaker implanted in the postoperative period due to the heart block. There was no early and late mortality. Conclusion: Management of multiple ventricular septal defects is quite complex. Right atrial approach of ventricular septal defects closure is safe, simple and effective in closure of multiple ventricular septal defects.

Echocardiogram in predicting correctable shunts in ventricular septal defect patients associated with severe pulmonary hypertension

Background Echocardiogram is a simple and useful tool in disease assessment.In ventricular septal defect(VSD)patients with severe pulmonary hypertension(PH),it is difficult to judge whether further intervention is needed.Therefore,this study aimed to investigate the application of echocardiogram in predicting the severity of pulmonary hypertension and guide subsequent management.Methods This study included VSD patients who underwent right heart catheterization(RHC)examination in Guangdong Provincial People's Hospital from January2011 to December 2022.An estimated pulmonary artery systolic pressure(e PASP)higher than 60 mm Hg was defined as severe PH in this study.Logistic regression analysis and receiver operating characteristic curve(ROC)analysis were used.Results A total of 186 VSD patients with severe PH(e PASP more than 60 mm Hg)were included in this study,with 158 cases in the non-correctable group and 28 cases in the collectable group.In univariable logistic regression,left atrium dimension(LA),left ventricular end-diastolic dimension(LVDd),left ventricular end-systolic dimension(LVDs),peak velocity of the pulmonary valve(PV),peak velocities from the early phase of the mitral inflow(MVE),bidirectional shunting and pericardial effusion were associated with a correctable shunt.When adjusted in multivariable model,only PV and bidirectional shunting remained statistically significant.The ROC curve found that PV exhibits good discriminative ability for correctable shunt(AUC[area under the curve]:0.779,95%CI:0.676-0.871)with a cut-off value of 1.465 m/s.The predictive performance of bidirectional shunting was not satisfactory,with a low AUC of 0.669(95%CI:0.571-0.766).Conclusions PV and bidirectional shunting are simple and clinically available parameters from echocardiogram in predicting PH severity,which not only avoids the repeated unnecessary cardiac catheterization,but also provides a reference basis for follow-up evaluation.

Impact of right ventricle to pulmonary artery connection and systemic-to-pulmonary artery shunt surgery on promoting the development of pulmonary vasculature in patients with pulmonary atresia with ventricular septal defect

Background Pulmonary atresia with ventricular septal defect(PA/VSD)is a rare and complex congenital heart disease(CHD).The optimal palliative surgical strategy for pulmonary atresia with ventricular septal defect(PA/VSD)in neonates and young infants is controversial.Surgery mainly includes the following two options,right ventricle to pulmonary artery connection(RV-PA)and systemic-to-pulmonary artery shunt surgery(SPS).Objectives:To determine the impact of the right ventricle to pulmonary artery connection or systemic-to-pulmonary artery shunt surgery as the initially palliated surgical strategy on promoting the development of pulmonary vasculature in patients with pulmonary atresia and ventricular septal defect(PA/VSD).Methods:From January 2010 to December 2019,104 patients with PA/VSD in Guangdong Cardiovascular Institute who underwent right ventricle to pulmonary artery connection or systemic-to-pulmonary artery shunt surgery as the initially palliated surgical strategy to promoting the development of pulmonary vasculature were identified and enrolled in this retrospective study.The cohort was divided into two groups:group-Right Ventricle to Pulmonary Artery Connection(RV-PA),who underwent initial palliation with staged repair(n=51),and group-Systemic-to-Pulmonary Artery Shunt(SPS)(n=53).Preoperative and postoperative the development of pulmonary vasculature data have been collected and compared.Results:Before the surgery,the Mc Goon Ratio of the RV-PA group significantly high than and the SPS group(P<0.05).After the surgery,in the RV-PA group,the Nakata index,Mc Goon Ratio score significantly increased during the interstage period(P<0.01).At the meantime,the HCT,RBC and HB significantly reduced in the RV-PA group compared with the SPS group(P<0.05).The shunt group performed better in these areas:length of hospital stay,ACC time and CPB time(P<0.05).Conclusion:Compared with systemic-to-pulmonary artery shunt,right ventricle to pulmonary artery connection is more effective to promote the development of the pulmonary vasculature for pulmonary atresia with ventricular septal defect.

Midterm results of diagnostic treatment and repair strategy in older patients presenting with nonrestrictive ventricular septal defect and severe pulmonary artery hypertension

Background Congenital heart disease with severe pulmonary arterial hypertension （SPAH）,previously thought to have irreversible pulmonary vascular disease （PVD）,has been recently successfully corrected using diagnostic treatment and repair strategy by our surgery team.This study aimed to evaluate the midterm results of a selected cohort of older patients with nonrestrictive ventricular septal defect （VSD） and SPAH using diagnostic treatment and repair strategy.Methods The records of 56 patients older than six years with nonrestrictive VSD and SPAH undergoing diagnostic treatment and repair strategy from 2006 to 2012 were retrospectively reviewed.All patients received advanced pulmonary arterial hypertension （PAH） therapy and radical repairs were performed when transcutaneous oxygen saturation （SPO2） increased up to 93％.Results There were no operative deaths.SPO2 and baseline six-minute walk test （SMWT） distance of all selected patients increased significantly and mean pulmonary artery pressure （MPAP） regressed significantly after diagnostic treatment and at late follow-up （P ＜0.01）.The incidence of late postoperative PAH was seen in six （10.7％） patients and by Logistic regression analysis,early postoperative PAH was an independent risk factor related to late postoperative PAH.Conclusions Diagnostic treatment and repair strategy was effective and safe for treatment of nonrestrictive VSD and SPAH and the midterm results were excellent.Diagnostic treatment strategy was effective in assessing the reversibility of SPAH in older patients associated with nonrestrictive VSD and the PVD in these selective patients is generally reversible.

Pulmonary Arterial Hypertension Medical Management of the Adult Patient with Congenital Heart Disease

Congenital heart disease(CHD)-associated pulmonary arterial hypertension(PAH)includes a heterogeneous patient population that can be characterized by the underlying cardiac malformation.CHD-associated PAH has an estimated prevalence of 5– 10% in adult patients,with an increasing number of patients surviving to adulthood because of advances in the surgical management and the development of pulmonary arterial hypertension(PAH)-targeted pharmacotherapy.Although limited data exist,targeted PAH pharmacotherapy has proven to be benefi cial in patients with CHD-associated PAH,with observed improvement in functional class,increase in exercise capacity,and improvement in quality of life and cardiopulmonary hemodynamics.Additionally,there has been increasing interest in the“treat-to-close”strategy.PAH-targeted pharmacotherapy may be used to optimize cardiopulmonary hemodynamics so as to improve patients’operability in repairing the cardiac defect.Although there have been signifi cant advances in the management of this disease state in the past 2 decades,mortality remains high,and ongoing clinical trials are needed to better understand the treat-to-close strategy.

Diagnostic chest X-ray in atrial septal defects

A 66-year-old woman with lower limb edema and exertional dyspnea presented to the emergency department.Posteroanterior chest X-ray revealed a Fleischner’s sign in both lungs(Figure 1A)suggesting a pulmonary hypertension.A subsequent transthoracic echocardiogram revealed a 24 mm diameter secundum atrial septal defect(ASD)(Figure 1B)with a dilated right heart and a mean pulmonary pressure of 68 mmHg.Fleischner’s sign refers to a prominent central pulmonary artery that can be commonly caused either by pulmonary hypertension or acute pulmonary embolism.This radiological finding is very rare in patients with secundum ASD unless the defect remains undiagnosed till the elderly.Chest X-ray remains fundamental in making diagnosis of cardiovascular disease.

Open Surgery for Retrieval of Patent Foramen Ovale Closure Device Embolized into Right Pulmonary Artery

Percutaneous Patent Foramen Ovate PFO/Atrial Septal Defect (ASD) closure has become an increasingly simplified procedure over the past decade. The main advantages of a percutaneous approach include avoidance of surgery, short procedure time and hospital stay. Device embolization is seen rarely but it can be fatal. We report this complication following a percutaneous PFO closure in a 44-year-old man. The device was embolized into the distal part of the right pulmonary artery. We removed the device surgically and closed the PFO/ASD.

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 （P=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.

Occult anomalous origin of the left coronary artery from the pulmonary artery with atrial septal defect initially visualized by transthoracic echocardiography

Anomalous origin of left coronary artery from the pulmonary artery （ALCAPA） is a rare congenitalcardiac malformation. Here we report a case of ALCAPA with atrial septal defect （ASD） initially visualized by transthoracic echocardiography, subsequently confirmed by the coronary angiography.

HD-Flow联合STIC在胎儿肺动脉闭锁合并室间隔缺损诊断中的应用价值

目的:探讨高分辨率血流成像(HD-Flow)联合时间-空间复合成像(STIC)诊断胎儿肺动脉闭锁合并室缺(PA-VSD)的临床价值。方法:分析经胎儿超声心动图诊断的44例PA-VSD胎儿心脏二维、HD-Flow联合STIC图像,评估HD-Flow联合STIC在PA-VSD不同分型诊断中的效能,并探讨PA-VSD胎儿动脉导管内径与左、右肺动脉内径相关性。结果:HD-Flow联合STIC诊断44例PA-VSD,其中A型42例,C型2例。HD-Flow联合STIC能够清晰显示胎儿肺动脉及其分支血管及动脉导管血流;Pearson相关性分析表明A型动脉导管内径和左、右肺动脉内径均呈正相关(r=0.507、0.404,P<0.01)。结论:HD-Flow联合STIC能够明确诊断PA-VSD并分型,有较大的临床应用价值,A型PA-VSD胎儿动脉导管内径与左、右肺动脉内径存在正相关。