Isolated atrial septal defect with pulmonary vascular obstructive disease--long-term follow-up and prediction of outcome after surgical correction.

Abstract

We examined the cases of 702 patients found to have isolated atrial septal defect of the secundum or sinus venosus type at catheterization from 1953 to 1978. Forty patients (6%), 34 women and six men, had pulmonary vascular obstructive disease, with a total pulmonary resistance greater than 7 U/M2; of these patients 26 (mean age 47 years) underwent surgical closure and 14 (mean age 44 years) received medical treatment. All patients were followed for at least 4 years, with a median follow-up of 12 years. At the most recent follow-up, 17 of the 40 patients were dead. Of the 22 surgically treated patients with total pulmonary resistance less than 15 U/M2, 19 were alive with significant regression of symptoms. All four surgically treated patients with total pulmonary resistance greater than or equal to 15 U/M2 were dead. Of the five medically treated patients with total pulmonary resistance less than 15 U/M2, four had died, and one was alive with significant progression of symptoms. Of the nine medically treated patients with total pulmonary resistance greater than or equal to 15 U/M2, six had died and the three survivors had progression of symptoms. In the surgically treated group, the following variables correlated with survival: total pulmonary resistance (p < .00001), pulmonary arteriolar resistance (p < .00001), pulmonary-to-systemic resistance ratio ( p = .004), systemic arterial oxygen saturation (p = .005), and pulmonary arterial oxygen saturation (p = .007). In conclusion:(1) Atrial septal defect with high total pulmonary resistance is uncommon and predominates in adult female patients. (2) Total pulmonary resistance (or pulmonary arteriolar resistance) is the best predictor of surgical outcome. In patients with total pulmonary resistance less than 15 U/m2, surgical treatment is advised. (3) In patients with borderline total pulmonary resistance, the systemic arterial oxygen saturation provides a good prediction of surgical outcome. Circulation 76, No. 5, 1037-1042, 1987

Clinical and hemodynamic features of Eisenmenger syndrome patients at the time of first admission: a tertiary referral-center experience

“…Bu olguların yaklaşık %4-8'lik kısmında ise altta yatan konjenital hastalığın yeri ile defektin boyutuna bağlı olarak pulmoner damar yatağında yapısal ve fonksiyonel değişiklikler gelişerek şantın ters dönmesi sonucu Eisenmenger sendromu (ES) gelişebilmektedir (1)(2)(3)(4)(5). İlk olarak 1897 yılında Avusturyalı Doktor Victor Eisenmenger tarafından geniş ventriküler septal defektli (VSD) bir olguda tanımlanarak, 1958 yılında Paul Wood tarafından ES olarak isimlendirilmiştir.…”

Congenital Heart Disease and Pulmonary Hypertension

“…The potential for reversibility is difficult to assess, and there is a paucity of information in this regard. 2 Most of the pathophysiologic processes in PAH-CHD seem similar to the pathogenesis implicated in the development of other forms of PAH. The pathogenesis of PAH is explained by a persistent high flow and pressure in the pulmonary vasculature that causes endothelial damage, leading to loss of endothelial barrier function and imbalance of vasoactive mediators that favor vasoconstriction, inflammation, thrombosis, cell proliferation, apoptosis, and fibrosis and result in pulmonary vascular remodeling and irreversible PAH.…”

“…1 Multiple studies have since expanded our understanding of the syndrome to include other congenital heart defects. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] CHD is one of the world's leading birth defects and pulmonary arterial hypertension (PAH) associated with CHD is one of the most common causes of morbidity and mortality in this group of patients. 20 …”

Can Pulmonary Vasodilator Therapy Expand the Operative Indications for Congenital Heart Disease?

“…[35][36][37][38] Rahimtoola, et al 35) reported poor operative outcomes when the peak pulmonary arterial pressure was > 60 mmHg, and suggested a cutoff value of Rp 640 dyne·s·cm -5 for operation. Dave, et al 36) reported poor operative outcomes when the peak pulmonary arterial pressure was > 40 mmHg, and suggested a cutoff value of Rp/Rs 0.2 for operation.…”

“…Dave, et al 36) reported poor operative outcomes when the peak pulmonary arterial pressure was > 40 mmHg, and suggested a cutoff value of Rp/Rs 0.2 for operation. Koizumi, et al 37) recommended that operation should not be performed if Rp/Rs > 0.4. Yamaki, et al 38) reported their detailed lung biopsy findings in patients with ASD, and strongly recommended open lung biopsy when the peak pulmonary arterial pressure is > 70 mmHg, mean pulmonary arterial pressure is > 44 mmHg, pulmonary-to-systemic pressure ratio is > 0.66, Rp/Rs is > 0.…”