Article

Isolated peripheral pulmonary artery stenoses in the adult.

Department of Cardiology, Children's Hospital, Boston MA 02115, USA.
Circulation (Impact Factor: 15.2). 05/1996; 93(7):1417-23.
Source: PubMed

ABSTRACT Isolated peripheral pulmonary artery stenosis (PPS) in the adult is rare and frequently unsuspected. We review in this article our experience with 12 adult patients with isolated PPS, half of whom had been previously diagnosed with chronic pulmonary thromboembolic disease.
The presentation, evolution, and management of 12 adults with isolated PPS, 17 to 51 years of age (mean, 36.2 +/- 9.7 years), were evaluated. Presenting symptoms were dyspnea and fatigue. Three patients had New York Heart Association (NYHA) functional class III or greater. Lung perfusion scans revealed multiple segmental abnormalities in flow distribution in all patients. Oxygen desaturation at rest was present in 4 patients. At catheterization, right ventricular (RV) pressure was suprasystemic in 2 patients, systemic in 1, and more than half-systemic in 7. All had multiple bilateral non-uniform stenoses in segmental and subsegmental arteries. Balloon pulmonary angioplasty (BPA) to decrease RV hypertension and improve pulmonary flow distribution was performed in 11 patients. After BPA, vessel diameter increased > 50% in 10 patients, distal pulmonary artery pressure increased > or = 30% in 6, and RV pressure decreased > 30% in 5. One patient died shortly after BPA as a result of pulmonary hemorrhage. Immediate procedural success was achieved in 9 of 11 patients. At a mean follow-up period of 52 +/- 32 months, 7 patients had sustained symptomatic improvement (NYHA class I-II).
We describe a severe syndrome of isolated PPS in the adult that mimics chronic pulmonary thromboembolic disease. Pulmonary hemodynamics and angiography are required for definitive diagnosis. BPA may offer these patients successful short-term reduction in RV hypertension and alleviation of symptomatology.

0 Bookmarks
 · 
60 Views
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Interventional catheterisation comprises performing a palliative or curative procedure during arterial or venous cardiac catheterisation. This procedure can be performed at any age, equally well in the newborn or adults affected by congenital cardiopathy: in order to definitively treat a congenital cardiopathy, unrecognised in infancy but diagnosed in adulthood, such as pulmonary valvular stenosis, coarctation of the aorta, persistent ductus arteriosus, ostium secundum type interatrial communication, coronaro-cardiac fistula, pulmonary arterio-venous fistula, and patent foramen ovale; in order to improve the haemodynamic state of a complex congenital cardiopathy, for example by creating an interatrial communication, or by opening the pulmonary route in a complex cardiopathy which cannot be completely repaired; in order to complete a surgical procedure, to treat recurrence of peripheral pulmonary stenosis or recurrence of coarctation, and to embolise the systemic vessels leading off the aorta or veno-venous anastomoses after cavopulmonary intervention. Paediatric cardiology can lead to occlusion of a left-right or right-left shunt with different devices, alleviation of an arterial or venous valvular stenosis with dilatation catheters, or implantation of endoprostheses in arterial or venous stenoses.
    Archives des maladies du coeur et des vaisseaux 12/2002; 95(11):1027-34. · 0.40 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Selective right pulmonary arteriography and 3-dimensional computed tomography revealed multiple severe stenoses of the peripheral pulmonary artery associated with poststenotic aneurysms in a 65-year-old woman. She was referred to the hospital for evaluation of dry cough, gradually increasing dyspnea and multiple nodular shadows on a chest radiograph. Echocardiography and cardiac catheterization showed severe pulmonary hypertension, though other structural heart diseases or well-characterized congenital syndromes were ruled out. She was diagnosed as isolated peripheral pulmonary artery branch stenosis. Recent advances in CT technology enable a less-invasive assessment of pulmonary artery, and can be useful in the management of pulmonary arterial hypertension.
    Internal Medicine 01/2010; 49(17):1895-9. · 0.97 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Although pulmonary thromboendarterectomy is increasingly successful for the definitive treatment of chronic thromboembolic pulmonary hypertension (CTEPH), not all patients have surgically accessible disease. Others are poor surgical candidates because of comorbid illness. Therefore, for selected patients, we defined and implemented an alternative interventional strategy of balloon pulmonary angioplasty (BPA). Eighteen patients (mean age, 51.8 years; range, 14 to 75 years) with CTEPH underwent BPA; they averaged 2.6 procedures (range, 1 to 5) and 6 dilations (range, 1 to 12). Selection of pulmonary artery segments for dilation required (1) complete occlusion, (2) filling defects, or (3) signs of intravascular webs. After an average of 36 months of follow-up (range, 0.5 to 66 months), the average New York Heart Association class improved from 3.3 to 1.8 (P:<0.001), and 6-minute walking distances increased from 209 to 497 yards (P:<0.0001). Pulmonary artery mean pressures decreased from 43.0+/-12.1 to 33. 7+/-10.2 mm Hg (P:=0.007). Eleven patients developed reperfusion pulmonary edema; 3 required mechanical ventilation. BPA reduces pulmonary artery hypertension in patients with CTEPH and is associated with long-term improvement in New York Heart Association class and 6-minute walking distances. BPA is a promising interventional technique that warrants randomized comparison with medical therapy in CTEPH patients who are not surgical candidates.
    Circulation 02/2001; 103(1):10-3. · 15.20 Impact Factor