IV-2 A new method of creation of atrial septal defect. Three surgical experiences of ASD creation and pulmonary artery banding in double outlet right ventricle with pulmonary hypertension

Uncorrected atrial septal defect undergoes right ventricle chronic volume overload which may lead to pulmonary hypertension and Eisenmenger Syndrome. The soluble suppression of tumorigenicity-2 is a left ventricle strain biomarker; however, its role in right ventricle strain is unclear. This study aimed to investigate the implication of serum soluble suppression of tumorigenicity-2 in adult uncorrected atrial septal defect. This was a cross-sectional study. We enrolled 81 adult uncorrected secundum atrial septal defect patients. Clinical and hemodynamic data were collected. Serum samples were withdrawn from the pulmonary artery during right heart catheterization. Serum soluble suppression of tumorigenicity-2 and NT-proBNP levels were measured. Subjects were divided into three groups based on clinical and hemodynamic severity. The correlation of soluble suppression of tumorigenicity-2 with patients' data and comparison among groups were analyzed. A p value <0.05 was considered statistically significant. Results showed that, there were significant correlations between serum soluble suppression of tumorigenicity-2 and mean pulmonary artery pressure ( r = 0.203, p = 0.035) and right ventricle end-diastolic diameter ( r = 0.203, p <0.05). Median serum soluble suppression of tumorigenicity-2 level was incrementally increased from group I (atrial septal defect and no-pulmonary hypertension), group II (left-to-right atrial septal defect and pulmonary hypertension), to group III (Eisenmenger Syndrome): (17.4 ng/mL, 21.8 ng/mL, and 29.4 ng/mL, respectively). A post-hoc analysis showed that serum soluble suppression of tumorigenicity-2 level was significantly different between groups I and III ( p = 0.01). Serum N terminal pro brain natriuretic peptide (NT-proBNP) level was consistently associated with worse clinical and hemodynamic parameters. No correlation was found between serum soluble suppression of tumorigenicity-2 and NT-proBNP level. In conclusion, serum soluble suppression of tumorigenicity-2 level had significant positive correlation with mean pulmonary artery pressure and right ventricle end-diastolic diameter in uncorrected secundum atrial septal defect patients. Higher serum soluble suppression of tumorigenicity-2 level was associated with the presence of pulmonary hypertension and Eisenmenger Syndrome in uncorrected secundum atrial septal defect patients.

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Association between serum soluble ST2 level and right ventricle systolic function on pulmonary hypertension due to atrial septal defect

Journal of thee Medical Sciences (Berkala Ilmu Kedokteran) ◽

10.19106/jmedsci005203202005 ◽

2020 ◽

Vol 52 (03) ◽

Author(s):

Firandi Saputra ◽

Anggoro Budi Hartopo ◽

Hariadi Hariawan ◽

Dyah Wulan Anggrahini ◽

Lucia Kris Dinarti

Keyword(s):

Pulmonary Hypertension ◽

Right Ventricle ◽

Atrial Septal Defect ◽

Septal Defect ◽

Systolic Function ◽

Soluble St2

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Massive Pulmonary Artery Thrombosis, Pulmonary Hypertension and Untreated Atrial Septal Defect

Cardiology ◽

10.1159/000047421 ◽

2002 ◽

Vol 97 (1) ◽

pp. 53-54 ◽

Cited By ~ 3

Author(s):

Nobukazu Ishizaka ◽

Norihide Kage ◽

Haruko Iida ◽

Shinsuke Mutoh ◽

Yasunobu Hirata ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Artery ◽

Atrial Septal Defect ◽

Septal Defect ◽

Artery Thrombosis

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Pulmonary Artery Thrombi Detected by Echocardiography in Patients with Pulmonary Hypertension Secondary to Atrial Septal Defect

European Journal of Echocardiography ◽

10.1053/euje.2001.0067 ◽

2001 ◽

Vol 2 (3) ◽

pp. 149-153

Author(s):

C Kaymaz

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Artery ◽

Atrial Septal Defect ◽

Septal Defect

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Is combined MDCT and echocardiography needed to guarantee accuracy in diagnosis and surgical planning of DORV and associated anomalies?

Egyptian Journal of Radiology and Nuclear Medicine ◽

10.1186/s43055-021-00677-w ◽

2022 ◽

Vol 53 (1) ◽

Author(s):

Samira Saraya ◽

Yousra Mahmoud Ahmad ◽

Ragab Hani Donkol ◽

Hazem Hamed Soliman ◽

Reem Ibrahim Ismail ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Right Ventricle ◽

Diagnostic Accuracy ◽

Surgical Planning ◽

Septal Defect ◽

Preoperative Assessment ◽

Double Outlet Right Ventricle ◽

Great Vessels ◽

Associated Malformations

Abstract Background The term of Double-outlet right ventricle (DORV) is used to describe a spectrum of complex congenital cardiac malformations where anomalies of the ventriculo-arterial alignment are noted where both great arteries (pulmonary artery and aorta) originate completely or predominantly from the right ventricle. The purpose of this study is to evaluate the diagnostic accuracy of electrocardiography (ECG) gated multidetector computed tomography (MDCT) in preoperative assessment of DORV and its associated malformations, providing key anatomic parameters that affect surgical planning. Results A total of 50 patients diagnosed by echocardiography with DORV referred from our institute between September 2018 and August 2020 were enrolled. The location of the ventricular septal defect (VSD) to the great vessels and the alignment of the great vessels to each other as well as other associated malformations that affect the surgical planning were assessed by MDCT. The patients were retrospectively classified into subgroups according to the relative positions of the great arteries, the relationship between the great arteries and the VSD, and the presence of associated malformations according to the classification of Society of Thoracic Surgeons database. The diagnostic value of MDCT and transthoracic echocardiography (TTE) in evaluation of associated cardiac malformations apart from double outlet right ventricle was compared in 20 patients, whom surgeries were performed and surgical results were obtained. Fallot type of DORV was the most common clinical type which represented 48% of the patients in which the VSD is committed to the aorta. The second most common clinical type was Taussig–Bing anomaly which represented 30% of the patients and the VSD was seen committed to the pulmonary artery. The atrio-ventricular septal defect (AVSD)/uni-ventricular type represented 16% of the patients in the current study, with pulmonary and systemic venous anomalies, heterotaxy syndrome and situs anomalies were commonly associated. A total of 53 malformations apart from DORV were found in 20 patients whom performed surgical interventions. MDCT detected different vascular and situs anomalies in those patients with 100% sensitivity and 100% positive predictive value compared to 71.4% and 94% respectively in TTE. On the other hand, in our study TTE was superior to MDCT for detection of intracardiac anomalies with 100% sensitivity and 100% accuracy compared to 87.5% and 95% respectively in MDCT. Conclusions Our study suggested that ECG gated MDCT serves as a rapid, noninvasive imaging modality with good spatial resolution and provides excellent diagnostic image quality and has satisfactory diagnostic accuracy in the preoperative assessment of DORV. It is superior to echocardiography in providing a comprehensive mapping of the anatomy of complex anomalies especially that of extra-cardiac structures such as aorta, systemic veins, pulmonary veins, and pulmonary arteries. However, it can miss tiny intracardiac malformations. So combining the results of MDCT and echocardiography would be beneficial to guarantee the accuracy of diagnosis of DORV.

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Severe pulmonary hypertension and reduced right ventricle systolic function associated with maternal mortality in pregnant uncorrected congenital heart diseases

Pulmonary Circulation ◽

10.1177/2045894019884516 ◽

2019 ◽

Vol 9 (4) ◽

pp. 204589401988451 ◽

Cited By ~ 3

Author(s):

Anggoro B. Hartopo ◽

Dyah W. Anggrahini ◽

Detty S. Nurdiati ◽

Noriaki Emoto ◽

Lucia K. Dinarti

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Mortality Rate ◽

Right Ventricle ◽

Maternal Mortality ◽

Atrial Septal Defect ◽

Congenital Heart ◽

Septal Defect ◽

Systolic Function

Background Pregnant uncorrected congenital heart disease patients, especially those who already developed pulmonary hypertension, have increased risk for maternal mortality. The pulmonary hypertension severity and right ventricle function may be associated with higher maternal mortality. The study aimed to investigate the mortality rate of pregnant uncorrected congenital heart disease and the impact of pulmonary hypertension severity on mortality. Methods This is the sub study of COngenital HeARt Disease in adult and Pulmonary Hypertension Registry. The data of pregnant uncorrected congenital heart disease patients were analyzed from registry database. The maternal mortality was recorded. The data of demography, clinics, obstetrics, and transthoracic echocardiography were collected. The factors that influenced maternal mortality were analyzed. A statistical significance was determined when p value < 0.05. Results From 2012 until 2017, there were 78 pregnant congenital heart disease patients. Of them, 56 patients were eligible for analyses. The majority of congenital heart disease was atrial septal defect (91.1%). The maternal mortality rate was 10.7% (6 of 56). Pulmonary hypertension occurred in 48 patients, therefore the maternal mortality rate among congenital heart disease-pulmonary hypertension with majority of atrial septal defect was 12.5% (6 of 48). Among nonsurvivors, 100% suffered from severe pulmonary hypertension as compared to survivors (56.0%), p = 0.041. Most nonsurvivors were Eisenmenger syndrome (83.3%), significantly higher compared to survivors (22.0%), p = 0.006. Nonsurvivors had significantly worsened WHO functional class, reduced right ventricle systolic function, and right heart failure. The modes of maternal death were severe oxygen desaturation (66.7%) and respiratory failure and sepsis (33.3%). Most of the maternal deaths occurred within 24 h postpartum period. Conclusion Maternal mortality rate among pregnant uncorrected congenital heart disease with majority of atrial septal defect was 10.7% and among congenital heart disease-pulmonary hypertension with majority of atrial septal defect was 12.5%. Factors related with maternal mortality were severe pulmonary hypertension, Eisenmenger syndrome, and reduced right ventricle systolic function.

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