Pulmonary wedge angiography for prediction of pulmonary vascular disease in down syndrome

Abstract

We performed high resolution pulmonary wedge angiography (PWA) and conventional hemodynamics to predict the reversibility of structural pulmonary vascular disease. Sixty-one pulmonary wedge angiograms were performed on 41 patients with intracardiac shunts and Down syndrome (median age 8 months). Balloon occlusion wedge angiograms were analyzed for (1) monopedial branches from the distal 10 mm of muscular arteries, (2) capillary blush, (3) tapering indices, and (4) tortuosity. Twenty-five patients had open lung biopsy, graded by the Health Edwards classification, and analyzed morphometrically. Pulmonary vascular resistance of > or = 6 units was 100% sensitive and 94% specific for Heath Edwards Grade III-IV. A monopedial count < 3 vessels was 83% sensitive and 100% specific for Heath Edwards Grade III-IV. Abnormal capillary blush was 83% sensitive and 69% specific for Heath Edwards Grade III-IV. Tapering indices and tortuosity showed no significant correlation with lung biopsy. A combination of pulmonary vascular resistance < 6 units, monopedial count > or = 3, and normal capillary blush was 100% sensitive and specific for Heath Edwards Grade 0-II, and a combination of pulmonary vascular resistance > or = 6 units, monopedial count < 3, and abnormal capillary blush was 100% sensitive and specific for Heath Edwards Grade III-IV. Using the 3 criteria, Heath Edwards Grade was accurately predicted in 17 patients. In 4 patients, only 2 criteria were available. Morphometric analysis showed an inverse relationship between the lowest monopedial count and the number of occlusive vessels per cm of tissue, r = -0.74 p < 0.001. Arteries showing intimal and/or medial thickening causing > 90% luminal narrowing were scored as "occlusive." These results show that when the hemodynamic and pulmonary wedge angiography data are concordant, the structural changes of pulmonary vascular disease can be accurately predicted and lung biopsy may be avoided.

Features and Outcomes of Methamphetamine-associated Pulmonary Arterial Hypertension

“…Pulmonary wedge angiography was obtained by placing the pulmonary artery catheter in the wedge position with an inflated balloon. A sandwich solution of 5 ml iodinated contrast followed by 4-5 ml of heparinized saline was hand injected, the balloon gently deflated, and images captured with routine cineangiography (13,14). Demographic, clinical, hemodynamic, and outcomes data were captured in the Vera Moulton Wall Center Pulmonary Hypertension Database (see details in the online supplement).…”

Shortening fraction of the right ventricle: a comparison between euploid and trisomy 21 fetuses at week 11 to week 13 and 6 days of gestation

“…Furthermore, postnatally the pulmonary circulation in individuals with Down syndrome is often significantly affected by changes in the pulmonary vasculature. Increased pulmonary resistance is present in a large percentage of these individuals even at a very young age (Yamaki et al , 1983; Wilson et al , 1993; Kawai et al , 1995; Suzuki et al , 2000; Shah et al , 2004; Cua et al , 2007). This appears to be due to both primary changes in these vessels (persistence of fetal double capillary network, reduction of the cross‐sectional area of the vascular bed, thin tunica media in the pulmonary arteries, and impaired endothelial function) (Chi, 1975; Hals et al , 1993; Yamaki et al , 1993; Cappelli‐Bigazzi et al , 2004) and secondary to a reduction in the number of alveoli (Cooney and Thurlbeck, 1982).…”

Síndrome de Down y cardiopatías congénitas con hipertensión pulmonar severa. Resultados quirúrgicos

“…Wilson (28) en un estudio de 41 casos con Síndrome de Down y cardiopatías de este tipo detectó que en los pacientes con Resistencia Vascular Pulmonar (RVP) mínima mayor o igual a 6 Unidades las lesiones encontradas fueron grados III-IV con sensibilidad del 100% y especificidad del 94%. Nagata (18) por el contrario considera en base a sus hallazgos que existen pacientes sobre todo menores de un año con resultados del cateterismo muy contrarios a la reversibilidad de la HTP en todos sus parámetros en que la biopsia cualitativa plantea lesiones regresivas y la evolución posterior a la cirugía es favorable, al igual que asevera Bush (29) en sus trabajos.…”

“…Wu (31) tuvo los mismos resultados en 10 pacientes con Defecto de Septación AV completo, tanto en lo referente a las presiones pulmonares previas a la cirugía como a las posteriores, ya que en estas últimas hubo respuesta favorable con el decursar de su evolución, pero la edad quirúrgica promedio fue de menos de un año.. Wilson (28) concluye en su trabajo que la reversibilidad de los cambios vasculares pulmonares no es sinónimo de supervivencia postoperatoria inmediata pues hubo CHTPA fatales en el 20% de sus casos. Sakamoto (32) presenta la casuística de cinco pacientes operados de CIV cuyas edades oscilaron entre dos y 48 meses que tuvieron en común lesiones grado II de Heath y Edwards, RVP elevadas y relación Gasto Pulmonar-Gasto Sistémico (QP/QS) no muy elevados en el cateterismo previo, y en los cuales las CHTPA se asociaron con atelectasia y derrame pleural, y el uso de Prostaciclina endovenosa fue efectivo en el control de las crisis, teniendo una evolución favorable tardía de la hipertensión pulmonar.…”