Pulmonary Stenosis with Intact Ventricular Septum: Clinical and Hemodynamic Correlation

肺動脈瓣狹窄：臨床學與血行動力學的配合

蘇文鉁(Wen-Jen Su) ； 馬芳傑(Fung-Jeih Ma)

《Acta Paediatrica Sinica》 24卷4期 (1983/12) Pp. 246-253

摘要

肺動脈瓣狹窄可被定義為：解剖學上看到右心室出口有一狹隘或生理學上顯示右心室與肺動脈間有一壓力差距。這是一種較常見的先天性心臟病，手術預後良好。此篇的目的是將臨床所見與行動力學之嚴重性相配合，作為指引我們何者需要進一步作心導管檢查。病人總數30人是經由心導管證實肺動脈瓣狹窄且心室中隔完整。我們依照其理學，胸部X光，心電圖，超音波心圖，血行動力學及血管攝影術的結果作分析討論。大部份病人為無症狀，若有症狀以疲勞及運動時氣喘為最常見解。發紺，心衰竭，顫動(thrill)，心雜音在4度以上且P2變小或聽不見表示為嚴重患者，胸部X光除少數壓力差距較大之患者外大都正常，肺動脈的狹窄後擴大(Poststenotic dilatation)在任何程度均可見。由心電圖本身或者利用臨床症狀，理學檢查所得合併心電圖(Multivariate analysis)所推測之壓力差距與經心臟導管檢查所得之資料相比，其相關系數均為0.75。此外，超心波心圖上明顯的“a”dip，肺動脈瓣的早期開放及右心室前壁肥厚亦可表示其嚴重程度。

並列摘要

Thirty patients with pulmonary stenosis (PS) were studied retrospectively with the purpose of correlating the symptoms and physical findings, chest X-ray, electrocardiograms, and echocardiograms with the cardiac catheterization data. According to the RV-PA pressure gradients (mmHg) (PG), they were divided into four groups: trivial (＜25 mmHg), mild (25-49) moderate (50-79) and severe (＞80). About one third of our patients were asymptomatic. Fatigue and dyspnea were the most common symptoms. Cyanosis, congestive heart failure, systolic thrills, and grade 4 systolic murmur with diminished or absent P2 were mostly observed in the group with severe stenosis. Presence of QV1, RV1≥20, SV6≥15, RV1+SV6≥35, Inverted T on AVF and V(subscript 1-4) and ST depression occurred exclusively in patients who had a severe pressure gradient. Most patients except those with severe stenosis had no cardiac enlargement. Poststenotic dilatation of the pulmonary artery was observed in all of the four groups. The correlation coefficients between the estimated RV-PA pressure gradients (by multivariate analysis and electrocardiographic items) and the measured RV-PA pressures were significantly high (r=0.75). Prominent ”a” dip, presystolic full opening of the pulmonary valve, thickened right ventricular anterior wall and dilated right ventricle were observe mainly in the PS patients with severe RV-PA PG. Nine Ps patients were found to have interatrial shunts: six of them were right-to-left and the other three were left-to-right. Severe RV-PA PG with a small atrial septal defect was the characteristic findings of the former, whereas the latter had mild to moderate PG with a large atrial defect. All patients who had PG＞50 mmHg underwent surgery, and the postoperative results were satisfactory. Those not underwent surgery continued to have stable estimated PG during the period of our follow-up study.

並列關鍵字

pulmonary stenosis ； correlation of clinical and physiologic data