Atrial septal defect closure with an Amplatzer septal occluder fenestrated with a coronary stent in a child with pulmonary arterial hypertension

2012 ◽  
Vol 23 (5) ◽  
pp. 692-696 ◽  
Author(s):  
Gregory J. Skinner ◽  
Robert M. Tulloh ◽  
Andrew J. Tometzki ◽  
Ingram Schulze-Neick ◽  
Gareth J. Morgan
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arterial Pressure ◽  
Severe Disease ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Stent Insertion ◽  
Pulmonary Arterial

AbstractThe presence of an atrial septal defect in pulmonary hypertension has benefits and detractions. Even in idiopathic pulmonary arterial hypertension, a significant left-to-right shunt at atrial level may increase the pulmonary arterial pressure and exacerbate the disease. However, it is well recognised that the presence of an atrial communication may be protective in subgroups with severe disease, allowing maintenance of cardiac output during times of increased pulmonary resistance. In the present paper, we present the case of a young boy with significant idiopathic pulmonary arterial hypertension and an atrial septal defect. We report our technique of septal occlusion using a device to decrease left-to-right shunting with concomitant stent insertion in that device to maintain the potential for right-to-left shunting during times of high pulmonary arterial pressure.

scholarly journals Role of Endothelin-1 Receptor Antagonist in Reducing Perioperative Pulmonary Arterial Hypertension after Surgical Correction of Ventricular Septal Defect

2016 ◽  
Vol 9 (1) ◽  
pp. 55-59
Author(s):  
Rampada Sarker ◽  
ASMS Islam ◽  
SC Mandal ◽  
Kazi Abul Hasan ◽  
Manoz Kumar Sarker ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Ventricular Septal Defect ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Septal Defect ◽  
Pulmonary Arterial Pressure ◽  
Surgical Correction ◽  
Endothelin 1 ◽  
Pulmonary Arterial

Background: This study was designed to determine the role of oral ambrisentan, an endothelin-1 receptor, in reducing perioperative pulmonary arterial hypertension during surgical correction of ventricular septal defect.Methods: This study was carried out among 54 patients of ventricular septal defect with pulmonary arterial hypertension undergoing surgical correction. The patients were divided into two groups; study group received oral ambrisentan, an endothelin-1 receptor antagonist peri-operatively, starting one week before surgery. Pulmonary arterial pressure was measured by echocardiography, cardiac catheterization and directly from pulmonary artery during surgical procedure.Results: Pulmonary arterial pressure was reduced significantly in the group in which oral amrisentan was given perioperatively. There was further reduction of pulmonary arterial pressure at discharge from hospital and at one month follow-up.Conclusion: Oral ambrisentan can reduce pulmonary arterial pressure perioperatively in patients underwent surgical correction of ventricular septal defect.Cardiovasc. j. 2016; 9(1): 55-59

scholarly journals Heart and lung transplantation in pulmonary arterial hypertension related to congenital heart disease: an unusual indication

2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Rosaria Barracano ◽  
Heba Nashat ◽  
Andrew Constantine ◽  
Konstantinos Dimopoulos
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Pulmonary Vascular Disease ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Recurrent Haemoptysis

Abstract Background Eisenmenger syndrome is a multisystem disorder, characterised by a significant cardiac defect, severe pulmonary hypertension and long-standing cyanosis. Despite the availability of pulmonary hypertension therapies and improved supportive care in specialist centres, Eisenmenger patients are still faced with significant morbidity and mortality. Case presentation We describe the case of a 44-year-old woman with Eisenmenger syndrome secondary to a large secundum atrial septal defect. Her pulmonary vascular disease was treated with pulmonary vasodilators, but she experienced a progressive decline in exercise tolerance, increasing atrial arrhythmias, resulting in referral for transplantation. Her condition was complicated by significant recurrent haemoptysis in the context of extremely dilated pulmonary arteries and in-situ thrombosis, which prompted successful heart and lung transplantation. She made a slow recovery but remains well 3 years post-transplant. Conclusions Patients with Eisenmenger syndrome secondary to a pre-tricuspid lesion, such as an atrial septal defect have a natural history that differs to patients with post-tricuspid shunts; the disease tends to present later in life but is more aggressive, prompting early and aggressive medical intervention with pulmonary arterial hypertension therapies. This case illustrates that severe recurrent haemoptysis can be an indication for expediting transplantation in Eisenmenger syndrome patients.

scholarly journals Right ventricle performances with echocardiography and 99mTc myocardial perfusion imaging in pulmonary arterial hypertension patients

2018 ◽  
Vol 243 (9) ◽  
pp. 754-761
Author(s):  
Jie Liu ◽  
Lei Fei ◽  
Guang-Qing Huang ◽  
Xiao-Ke Shang ◽  
Mei Liu ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Myocardial Perfusion ◽  
Arterial Pressure ◽  
Right Ventricle ◽  
Arterial Hypertension ◽  
Pulmonary Arterial Pressure ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial

Right heart catheterization is commonly used to measure right ventricle hemodynamic parameters and is the gold standard for pulmonary arterial hypertension diagnosis; however, it is not suitable for patients’ long-term follow-up. Non-invasive echocardiography and nuclear medicine have been applied to measure right ventricle anatomy and function, but the guidelines for the usefulness of clinical parameters remain to be established. The goal of this study is to identify reliable clinical parameters of right ventricle function in pulmonary arterial hypertension patients and analyze the relationship of these clinical parameters with the disease severity of pulmonary arterial hypertension. In this study, 23 normal subjects and 23 pulmonary arterial hypertension patients were recruited from January 2015 to March 2016. Pulmonary arterial hypertension patients were classified into moderate and severe pulmonary arterial hypertension groups according to their mean pulmonary arterial pressure levels. All the subjects were subjected to physical examination, chest X-ray, 12-lead electrocardiogram, right heart catheterization, two-dimensional echocardiography, and technetium 99m (99mTc) myocardial perfusion imaging. Compared to normal subjects, the right heart catheterization indexes including right ventricle systolic pressure, right ventricle end diastolic pressure, pulmonary artery systolic pressure, pulmonary artery diastolic pressure, pulmonary vascular resistance, and right ventricle end systolic pressure increased in pulmonary arterial hypertension patients and were correlated with mean pulmonary arterial pressure levels. Echocardiography parameters, including tricuspid regurgitation peak velocity, tricuspid regurgitation pressure gradient, tricuspid annular plane systolic excursion and fractional area, right ventricle-myocardial performance index, were significantly associated with the mean pulmonary arterial pressure levels in pulmonary arterial hypertension patients. Furthermore, myocardial perfusion imaging was not observed in the normal subjects but in pulmonary arterial hypertension patients, especially severe pulmonary arterial hypertension subgroup, and showed potential diagnostic properties for pulmonary arterial hypertension. In conclusion, mean pulmonary arterial pressure levels are correlated with several right heart catheterization and echocardiography markers in pulmonary arterial hypertension patients; echocardiography and 99mTc myocardial perfusion can be used to evaluate right ventricle performance in pulmonary arterial hypertension patients. Impact statement In this study, we analyzed the clinical parameters for evaluating RV function, including right ventricle catheterization (RHC), echocardiography, and technetium 99m (99mTc) myocardial perfusion imaging (MPI) in normal Asian subjects and PAH patients ( n = 23 for each group). Our results demonstrated that six RHC indexes, four echocardiography indexes and MPI index were significantly altered in PAH patients and correlated with the levels of mean pulmonary arterial pressure. Importantly, we evaluated the diagnostic performance of MPI and found that MPI has a strong diagnostic accuracy in PAH patients. The findings from this study will be of interest to clinical investigators who make diagnosis and therapeutic strategies for PAH patients.

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