A CASE OF PULMONARY HYPERTENSION DUE TO ATRIAL SEPTAL DEFECT COMPLICATED BY OCCULT CHRONIC THROMBOEMBOLISM

The aim of the study was to review our experience with atrial septal defect (ASD) closure with a fenestrated patch in patients with severe pulmonary hypertension. Between July 2004 and February 2009, 16 patients with isolated ASD underwent closure with a fenestrated patch. All patients had a secundum type ASD and severe pulmonary hypertension. Patients ranged in age from 6 to 57 years (mean � SD, 34.9 � 13.5 years). The follow-up period was 9 to 59 months (mean, 34.5 � 13.1 months). The ranges of preoperative systolic and pulmonary arterial pressures were 63 to 119 mm Hg (mean, 83.8 � 13.9 mm Hg) and 37 to 77 mm Hg (mean, 51.1 � 10.1 mm Hg). The ranges of preoperative values for the ratio of the pulmonary flow to the systemic flow and for pulmonary arterial resistance were 1.1 to 2.7 (mean, 1.95 � 0.5) and 3.9 to 16.7 Wood units (mean, 9.8 � 2.9 Wood units), respectively. There was no early or late mortality. Tricuspid annuloplasty was performed in 14 patients (87.5%). The peak tricuspid regurgitation gradient and the ratio of the systolic pulmonary artery pressure to the systemic arterial pressure were decreased in all patients. The New York Heart Association class and the grade of tricuspid regurgitation were improved in 13 patients (81.2%) and 15 patients (93.7%), respectively. ASD closure in patients with severe pulmonary hypertension can be performed safely if we create fenestration. Tricuspid annuloplasty and a Cox maze procedure may improve the clinical result. Close observation and follow-up will be needed to validate the long-term benefits.

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

Journal of Congenital Cardiology ◽

10.1186/s40949-020-00047-7 ◽

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.

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Pulmonary Vascular Disease in Secundum Atrial Septal Defect with Pulmonary Hypertension

CHEST Journal ◽

10.1378/chest.89.5.694 ◽

1986 ◽

Vol 89 (5) ◽

pp. 694-698 ◽

Cited By ~ 32

Author(s):

Shigeo Yamaki ◽

Togo Horiuchi ◽

Makoto Miura ◽

Yasuyuki Suzuki ◽

Eiji Ishizawa ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Atrial Septal Defect ◽

Vascular Disease ◽

Septal Defect ◽

Pulmonary Vascular Disease ◽

Secundum Atrial Septal Defect

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ATRIAL SEPTAL DEFECT- INDUCED PARTIALLY REVERSIBLE SEVERE PULMONARY HYPERTENSION: WHAT SHOULD WE DO?

Journal of the American College of Cardiology ◽

10.1016/s0735-1097(16)31098-1 ◽

2016 ◽

Vol 67 (13) ◽

pp. 1097

Author(s):

Mugurel Valentin Bazavan ◽

Abdalla Hassan ◽

Maria Bazavan ◽

Susheela Hadigal ◽

Sorin Danciu

Keyword(s):

Pulmonary Hypertension ◽

Atrial Septal Defect ◽

Septal Defect ◽

Severe Pulmonary Hypertension

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Abstract 2130: Long-term Outcomes of Adult Atrial Septal Defect With Severe Pulmonary Hypertension After Surgical Closure

Circulation ◽

10.1161/circ.116.suppl_16.ii_461-c ◽

2007 ◽

Vol 116 (suppl_16) ◽

Author(s):

Jeong-Hoon Kim ◽

Duk-Hyun Kang ◽

Jong-Young Lee ◽

Jong-Min Song ◽

Tae-Jin Yun ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Elderly Patients ◽

Atrial Septal Defect ◽

Septal Defect ◽

Survival Rates ◽

Severe Pulmonary Hypertension ◽

Term Survival ◽

Surgical Closure

The benefits of surgical closure has been unclear in adult atrial septal defect (ASD) with severe pulmonary hypertension (PHT), and we tried to evaluate improvement of PHT and long-term survival after surgical closure compared to medical follow-up. Methods: From 1996 to 2006, we included a total of 71 adult ASD patients (age; 43±15 years) with severe PHT documented by echocardiography. The inclusion criteria were defined as ASD diameter > 15 mm, enlarged right ventricle, and the baseline peak velocity of tricuspid regurgitation (TR) ≥ 4.0 m/sec. We excluded 5 patients with Eisenmenger syndrome documented by cardiac catheterization. Surgical closure was performed on 55 patients (OP group) and the remaining 16 patients were followed up medically (MED group). The improvement of PHT was defined as TR velocity ≤ 3.5 m/sec on follow-up echo. Results: Baseline characteristics and clinical results were compared between the two groups in table . There were no significant differences in terms of gender, ASD diameter, cardiac rhythm, and TR velocity, but the MED group was significantly older. During follow-up of 46±33 months, there were 5 deaths in the MED group and no operative or late death in the OP group, and the 5-year actuarial survival rate of the OP group was significantly higher than the MED group (58±15%, p<0.05). On subgroup analysis according to age, the OP group showed significantly better survival rates than the MED group (p<0.05) in elderly patients (age > 50). In the OP group, TR velocity was significantly decreased from 4.5±0.4 to 3.0±0.7 m/sec on follow-up echo, and improvement of PHT was observed in 47 (85%) patients. On multivariate analysis, female gender and lower baseline TR velocity were the significant independent predictors of improved PHT after surgery. Conclusions: In adult ASD with severe PHT, surgical closure can be safely performed and improve PHT effectively. Especially in elderly patients, ASD closure is significantly related with the better survival rates.

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