Two-Stage Procedure for Pulmonary Vascular Obstructive Disease in Down Syndrome With Congenital Heart Disease

Ohashi, Naoki; Matsushima, Masaki; Maeda, Masanobu; Yamaki, Shohei

doi:10.1253/circj.70.1446

Cited by 8 publications

(8 citation statements)

References 23 publications

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“…The incidence and severity of PAH did not significantly differ among patients with Rastelli type C AVSD, as compared with people without DS . A study of lung biopsy results for patients with PAH and pulmonary artery banding reported greater histologic improvement and better prognosis when two‐stage surgical procedures were selected…”

Section: Incidence Of Dsmentioning

confidence: 88%

Clinical characteristics of pulmonary arterial hypertension associated with Down syndrome

Saji

2014

Pediatrics International

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The genetic abnormalities associated with Down syndrome (DS) are still being identified. Few studies have examined the roles of CRELD1 and GATA4 in cardiac abnormalities or their association with pulmonary artery histopathology. Children with DS have an elevated risk of pulmonary arterial hypertension (PAH). This increased risk is likely mainly due to genetic background, the structural characteristics of the pulmonary vascular wall, and certain heart diseases and partly due to pulmonary hypoplasia, upper and lower airway obstructive diseases, chronic infection, and neuromuscular underdevelopment. Exposure to increased left-to-right shunt flow increases sheer stress on endothelium and may induce endothelial dysfunction followed by irreversible remodeling of pulmonary arteries. Pathologic changes include endothelial cell proliferation and thickening of the pulmonary arterial wall due to mechanical responses to the thinner medial smooth muscle cell layer, which includes underdevelopment of alveoli. Production of prostacyclin and nitric oxide is diminished in DS, but endothelin-1 and thromboxane are elevated. Perioperatively, patients with DS may experience pulmonary hypertensive crisis after intracardiac repair and prolonged PAH, and have a poorer response to nitric oxide inhalation. To better manage DS, it is crucial to systematically evaluate the systemic complications of DS. Cardiac catheterization data, particularly those regarding pulmonary arterial resistance, are essential in assessing severity and response to vasodilating agents, preventing postoperative crisis, and evaluating the possibility of intracardiac repair. Advanced therapy with pulmonary vasodilating agents appears effective. Operative risk is similar for individuals with and without DS, except among patients with a complete atrioventricular canal defect.

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Section: Incidence Of Dsmentioning

confidence: 88%

Clinical characteristics of pulmonary arterial hypertension associated with Down syndrome

Saji

2014

Pediatrics International

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“…A two-stage procedure helped prevent mortality due to obstructive pulmonary vascular lesions, indicating that this procedure was highly recommended [13]. …”

Section: Discussionmentioning

confidence: 99%

Pulmonary vascular disease associated with pulmonary hypertension in 445 patients: diagnosis from lung biopsy and autopsy

Yamaki¹

2012

Gen Thorac Cardiovasc Surg

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PurposeDiagnosis from lung biopsy or autopsy was performed in 445 patients with congenital (385) or acquired (60) heart disease from all over Japan. The purpose of this study is the presentation of these prospective data collections.MethodsOf the patients with congenital heart disease, 354 were biopsied to determine whether surgery was indicated. Decisions regarding surgery were based on the index of pulmonary vascular disease in simple cardiac anomalies or atrioventricular septal defects (AVSD). In total anomalous pulmonary venous connection (TAPVC), operative indication was determined by the degree of hypoplasia of small pulmonary arteries. Operability of Fontan procedure was based on the degree of residual medial hypertrophy after pulmonary artery banding.ResultsIn patients with simple cardiac anomalies, radical surgery was indicated in 166. Radical surgery was indicated in 50 patients with AVSD. In 26 patients with TAPVC, radical surgery was not indicated in 10. In 68 Fontan candidates, surgery was not indicated in 49. Among 7 patients with tetralogy of Fallot, 1 was not a surgical candidate. Of the 60 acquired heart disease patients, 16 had idiopathic pulmonary arterial hypertension and 36 had chronic thromboembolic pulmonary hypertension. In 6 patients, lung biopsy revealed pulmonary veno-occlusive disease; 2 patients had combined valvular disease.ConclusionThe cardiac surgeon, pediatric cardiologist, and cardiologist who requested diagnosis from lung biopsy or autopsy were gratified with the results.

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“…In the context of cardiac surgery, pulmonary artery banding (PAB) is associated with certain indicators, such as the multiple ventricular septal defects (VSD), 1 VSD with aortic coarctation (COA), 2 univentricular hearts with increased pulmonary blood flow, 3,4 to train the left ventricle before arterial switch operation in certain cases of transposition of the great arteries 5,6 and other rare indicators reported elsewhere. [7][8][9] Historically, PAB had been performed through three surgical approaches; (i) left lateral thoracotomy, (ii) median sternotomy, and (iii) left anterior thoracotomy, 10,11 based on the need for performing additional procedures and the preference of the surgical team.…”

Section: Discussionmentioning

confidence: 99%

Upper mini sternotomy approach for pulmonary artery banding: A single centre experience

Salem

Walley

Khaymaf

et al. 2021

Journal of Cardiac Surgery

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Background: Pulmonary artery banding (PAB) remains a crucial technique in modern cardiac surgery. Left lateral thoracotomy, median sternotomy, and left anterior thoracotomy are well-known approaches. With significant scarce reports addressing the application of the upper mini sternotomy approach for PAB, this study aims to share experience and report outcomes of patients operated upon using this approach and its impact on facilitating the redo surgery.

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Two-Stage Procedure for Pulmonary Vascular Obstructive Disease in Down Syndrome With Congenital Heart Disease

Cited by 8 publications

References 23 publications

Clinical characteristics of pulmonary arterial hypertension associated with Down syndrome

Clinical characteristics of pulmonary arterial hypertension associated with Down syndrome

Pulmonary vascular disease associated with pulmonary hypertension in 445 patients: diagnosis from lung biopsy and autopsy

Upper mini sternotomy approach for pulmonary artery banding: A single centre experience

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