Abstract 15542: Late Pulmonary Hypertension in Surgically Palliated D-transposition of the Great Arteries: A 55-year Review

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Blair Suter ◽  
Dylan Hall ◽  
Eric S Ebenroth ◽  
Larry W Markham ◽  
Michael Johansen
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Late Onset ◽  
Systolic Dysfunction ◽  
Left Lung ◽  
Treatment Strategies ◽  
Lung Hypoplasia ◽  
Pulmonary Vascular Disease ◽  
Complete Evaluation ◽  
Lost To Follow Up

Introduction: Pulmonary hypertension (PH) has been described following surgical palliation for transposition of the great arteries (D-TGA). While early pulmonary vascular disease is associated with late repair, the increasing prevalence of late PH has been observed following the intra-atrial baffle procedure (ABP), Mustard/Senning. A previous study from our institution identified patients with precapillary PH. More recent studies have described predominately postcapillary PH, suggesting multiple, differing mechanisms for this complication. Hypothesis: While heart failure is a common complication following ABP and is associated with postcapillary PH, we hypothesize that precapillary PH can be identified in a subset of patients. Methods: A retrospective descriptive study was performed at a single institution. Using 6 th World Symposium on Pulmonary Hypertension definitions. PH was defined as a mean pulmonary artery pressure >20mmHg. Etiology of PH was defined as precapillary (≥3 Wood units) versus postcapillary (pulmonary artery wedge pressure >15 mmHg). Results: We reviewed 157 D-TGA patients following ABP, finding 33 patients with PH. Mean age at last evaluation was 36.6 (14-54) years. Current condition: 22 alive, 7 dead, and 4 lost to follow-up. Etiology of PH: 10 precapillary, 8 postcapillary, 8 mixed, 2 borderline PH, and 2 did not undergo catherization. Additionally, 2 patients had pulmonary venous baffle obstruction and 1 had left lung hypoplasia. Of patients with recent imaging, 21 of 25 had systemic RV systolic dysfunction on echocardiogram or MRI. Conclusions: Late-onset PH in D-TGA following ABP is a significant long-term complication and warrants vigilant surveillance. This highlights the limitations of imaging and need for catheterization for complete evaluation. In this study, we observed precapillary PH in a majority of patients with PH, which differs from some previous studies. Identifying the etiology of PH could drastically alter treatment strategies and has implications for transplant consideration. Further studies are needed to identify the clinical attributes that contribute to this process.

scholarly journals Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis

2020 ◽  
Author(s):  
Guiomar Pinheiro ◽  
Ana Margarida Alves ◽  
Isabel Neves ◽  
Teresa Sequeira
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Hypoplasia ◽  
Left Lung ◽  
Left Pulmonary Artery ◽  
Pulmonary Infections ◽  
Rare Congenital Disorder ◽  
History Of ◽  
Pulmonary Artery Hypoplasia

Pulmonary hypoplasia or agenesis is a rare congenital disorder that results in lung underdevelopment. This disease is usually found in children but rarely encountered in adults. We describe the case of an 84-year-old woman diagnosed with a unilateral pulmonary hypoplasia presenting simultaneously with left pulmonary artery hypoplasia. Due to this condition, the patient had a lifelong history of pulmonary infections that resulted in several bronchiectases in the affected lung. Moreover, the pulmonary artery hypoplasia led to the development of pulmonary hypertension and collateral circulation causing hemoptysis, giving rise to the patient attending the emergency department. Although we were able to medically manage the hemoptysis, it can be fatal and require surgical intervention. Hence, an early diagnosis is essential so that appropriate follow-up and prompt prevention and treatment of complications, such as pulmonary infections, hemoptysis and pulmonary hypertension, are achieved.

Pulmonary hypertension and arterial changes in calves with a systemic-to-left pulmonary artery connection

1994 ◽  
Vol 77 (2) ◽  
pp. 867-875 ◽  
Author(s):  
J. W. Fasules ◽  
F. Tryka ◽  
C. W. Chipman ◽  
S. H. Van Devanter
Keyword(s):  
Pulmonary Artery ◽  
Structural Changes ◽  
Heart Defects ◽  
Left Lung ◽  
Left Pulmonary Artery ◽  
Pulmonary Vascular Disease ◽  
Vascular Changes ◽  
Small Vessels ◽  
Arteriolar Diameter ◽  
Increased Pressure

The pathogenic mechanisms by which increased pressure and flow lead to pulmonary vascular disease are poorly understood, especially in newborns. To study the pathophysiological correlations and timing of the development of structural changes in response to high flow in nonhypoxic neonates, a model of high pulmonary flow was developed in newborn calves by anastomosis of the isolated left pulmonary artery (LPA) to the aorta. LPA pressure and flow increased acutely. LPA pressure reached near-systemic levels by 10 wk, whereas LPA flow was maximally increased at 1 mo before decreasing in several calves. Right pulmonary arterial pressure remained normal, and ventricular hypertrophy did not develop. Morphometric evaluation of the left lung demonstrated decreased arteriolar diameter, increased medial thickness, muscularization of arterioles at the bronchoalveolar junction, luminal obliteration of small arteries, and dilation lesions. The LPA pressure and vascular changes were greater and developed over a shorter time period than did prior models of nonhypoxic flow-induced pulmonary vascular changes. Lesser degrees of decreased arteriolar diameter and muscularization of small vessels were seen in the right lung, indicating a difference in the vascular response to moderately increased flow vs. increased pressure and flow. Thus, calves with an isolated LPA-to-aortic anastomosis simulate the hemodynamic and pulmonary vascular changes seen in newborns with congenital heart defects. Such calves may serve as models to assess effects of mechanical stresses on a newborn's vasculature.

scholarly journals LATE ONSET PULMONARY HYPERTENSION IN A PATIENT WITH UNILATERAL ABSENCE OF THE PULMONARY ARTERY AND PULMONARY APLASIA

CHEST Journal ◽  
2020 ◽  
Vol 158 (4) ◽  
pp. A1643
Author(s):  
Evan Wasserman ◽  
Karthik Gonuguntla ◽  
Nikola Perosevic
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Late Onset

scholarly journals The Role of Peroxisome Proliferator-Activated Receptors in Pulmonary Vascular Disease

PPAR Research ◽  
2007 ◽  
Vol 2007 ◽  
pp. 1-10 ◽  
Author(s):  
Rachel E. Nisbet ◽  
Roy L. Sutliff ◽  
C. Michael Hart
Keyword(s):  
Pulmonary Hypertension ◽  
Vascular Disease ◽  
Vascular Dysfunction ◽  
Treatment Strategies ◽  
Underlying Disease ◽  
Nuclear Hormone Receptor ◽  
Pulmonary Vascular Disease ◽  
Peroxisome Proliferator ◽  
Peroxisome Proliferator Activated Receptors ◽  
And Function

Peroxisome proliferator-activated receptors (PPARs) are ligand-activated transcription factors belonging to the nuclear hormone receptor superfamily that regulate diverse physiological processes ranging from lipogenesis to inflammation. Recent evidence has established potential roles of PPARs in both systemic and pulmonary vascular disease and function. Existing treatment strategies for pulmonary hypertension, the most common manifestation of pulmonary vascular disease, are limited by an incomplete understanding of the underlying disease pathogenesis and lack of efficacy indicating an urgent need for new approaches to treat this disorder. Derangements in pulmonary endothelial-derived mediators and endothelial dysfunction have been shown to play a pivotal role in pulmonary hypertension pathogenesis. Therefore, the following review will focus on selected mediators implicated in pulmonary vascular dysfunction and evidence that PPARs, in particular PPARγ, participate in their regulation and may provide a potential novel therapeutic target for the treatment of pulmonary hypertension.

scholarly journals A neonate with left pulmonary artery thrombosis and left lung hypoplasia: a case report

2010 ◽  
Vol 4 (1) ◽  
Author(s):  
Nahed O ElHassan ◽  
Christi Sproles ◽  
Ritu Sachdeva ◽  
Sadaf T Bhutta ◽  
Joanne S Szabo
Keyword(s):  
Case Report ◽  
Pulmonary Artery ◽  
Left Lung ◽  
Left Pulmonary Artery ◽  
Lung Hypoplasia ◽  
Artery Thrombosis

scholarly journals Segmental Pulmonary Hypertension in Children with Congenital Heart Disease

Medicina ◽  
2020 ◽  
Vol 56 (10) ◽  
pp. 492
Author(s):  
Bibhuti B. Das ◽  
Benjamin Frank ◽  
Dunbar Ivy
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Multimodality Imaging ◽  
Heart Diseases ◽  
Heart Defects ◽  
Pulmonary Atresia ◽  
Pulmonary Vascular Disease ◽  
Variable Degree ◽  
Branch Pulmonary Artery Stenosis

Segmental pulmonary hypertension is a complex condition in children that encompasses many congenital heart diseases including pulmonary atresia with ventricular septal defect, hemitruncus/truncus arteriosus with branch pulmonary artery stenosis, unilateral absent pulmonary artery, and several post-tricuspid shunt lesions. Multimodality imaging is required to confirm and assess pulmonary vascular disease in subjects with major aorto-pulmonary collaterals. We describe 3 children with complex congenital heart defects who have a variable degree of segmental pulmonary hypertension and discuss management strategies and the role of interventional and/or pulmonary hypertension targeted therapies.

scholarly journals Large Aorto-Pulmonary Window (APW) associated with severe pulmonary hypertension: A case report

2017 ◽  
Vol 1 (1) ◽  
pp. 29
Author(s):  
Abdul Wasay Khan
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Past History ◽  
Ductus Arteriosus ◽  
Pulmonary Vascular Disease ◽  
Type I ◽  
High Grade Fever ◽  
Surgical Closure

Background: APW is a rare congenital cardiac abnormality, accounting for 0.2% to 0.6% of patients. Hence the reported surgical experience is minimal. It is a     communication b/w pulmonary artery (PA) and the ascending aorta (Ao), in the presence of two separate semi lunar valves. Heart failure and irreversible pulmonary hypertension are the fearsome outcomes of untreated APW. Therefore, surgical  closure is the first-line curative choice. We present a case of young boy who presented late in age (his second decade of life) with severe signs and symptoms. Case   Presentation: A 12-year-old male patient from Baluchistan (balochi) presented with complaints of high grade fever for one week, palpitations since 12 days, along with past history of shortness of breath. On examination patient was anemic, cyanosed with raised JVP. On precordial examination apex beat was localized at the 6th     intercostal space shifted to mid-axillary line. S1 was normal but S2 was loud with prominent pulmonary component. Grade IV/VI continuous murmur was heard in the left upper sterna border. Chest X-ray showed cardiomegaly with prominent pulmonary artery (PA) and bilateral lung congestion. Echocardiography showed enlarged and hypertrophied right ventricle associated with dilated PA and a large APW was seen with severe pulmonary artery pressure (PAP) of 102 mmHg. Diagnosis of APW was confirmed on cardiac catheterization. Furthermore, cardiac CT was performed which showed type I APW with severe PH and large patent PDA (patent ductus  arteriosus). Patient then underwent repair of APW associated with reactive     pulmonary hypertension that had a complete repair utilizing a trans-aortic approach. PDA was also ligated during the same procedure. Patient symptoms remarkably  improved with a reduction PAP (40mmHg) after surgery and at 6 weeks follow-up. Follow-up echocardiography showed complete closure of the APW, and       pharmacological therapy was no longer required in the patient. Conclusion: APW should be suspected in any child, who comes with repeated chest infections and signs of congestive heart failure. It should be surgically corrected early, in order to prevent the development of obstructive pulmonary vascular disease. There is conflict of   interest.

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