Alveolar Capillary Dysplasia: Diagnostic Potential for Cardiac Catheterization

Hintz, Susan R.; Vincent, Julie; Pitlick, Paul T.; Fineman, Jeffrey R.; Steinhorn, Robin H.; Kim, Grace; Benitz, William Ε.

doi:10.1038/sj.jp.7200243

Cited by 18 publications

(13 citation statements)

References 15 publications

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“…We speculate that increased hemodynamic stress due to high pulmonary artery pressure or redistribution of pulmonary blood flow contribute to the persistence and hypertrophy of this vascular system in BPD, which has been observed with marked venous obstruction (32,(35)(36)(37). Whether or not the local production of vasoactive factors could contribute to the failure of IAAV to close after birth in preterm infants with severe BPD remains speculative.…”

Section: Discussionmentioning

confidence: 98%

“…Bronchial veins drain lobular, segmental, and peripheral bronchiole arteries and return blood into pulmonary veins (34). In severe pulmonary venoocclusive disorders, these veins often appear congested and prominent, as observed in misalignment of pulmonary veins with alveolar capillary dysplasia, and are postulated to serve as "decompressing pathways" (35)(36)(37). Our three-dimensional reconstruction studies show that prominent veins within the BPD lung connect pulmonary veins within the primary septa with pulmonary arteries and vessels of bronchial and periarterial microvascular plexuses (vasa vasorum) ( Figure 3; Video E2).…”

Section: Discussionmentioning

confidence: 99%

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Histologic Evidence of Intrapulmonary Anastomoses by Three-Dimensional Reconstruction in Severe Bronchopulmonary Dysplasia

2013

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Rationale: Bronchopulmonary dysplasia (BPD) is the chronic lung disease of infancy that occurs in premature infants after oxygen and ventilator therapy for acute respiratory disease at birth. Despite improvement in current therapies, the clinical course of infants with BPD is often characterized by marked hypoxemia that can become refractory to therapy. Preacinar anatomic and functional communications between systemic and pulmonary vascular systems has been established in fetal lungs, but whether increased intrapulmonary anastomotic vessels or their failure to regress after birth contributes to hypoxemia in preterm infants with BPD is unknown.Objectives: We sought to find histologic evidence of intrapulmonary anastomotic vessels in lungs of patients who died of severe BPD. Methods:We collected lung tissues from fatal BPD cases and performed histology, immunohistochemistry, and high-precision three-dimensional reconstruction techniques. Measurements and Main Results:We report histologic evidence of intrapulmonary vessels that bridge pulmonary arteries and veins in the distal lungs of infants dying with severe BPD. These prominent vessels appear similar to "misaligned pulmonary veins" described in the lethal form of congenital lung disorder, alveolar capillary dysplasia. Conclusions:We found striking histological evidence of precapillary arteriovenous anastomotic vessels in the lungs of infants with severe bronchopulmonary dysplasia. We propose that persistence or expansion of these vessels after premature birth provides the anatomic basis for intrapulmonary shunt and hypoxemia in neonates with severe bronchopulmonary dysplasia and may play a significant role in the morbidity and mortality of BPD.Keywords: intrapulmonary shunt; bronchopulmonary dysplasia; pulmonary circulation; vascular development

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Histologic Evidence of Intrapulmonary Anastomoses by Three-Dimensional Reconstruction in Severe Bronchopulmonary Dysplasia

2013

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“…The presence of delayed or absent capillary blush phase on pulmonary angiography has been reported in a small series of patients with ACD/MPV who underwent cardiac catheterization for evaluation of pulmonary hypertension (36). At present, this finding is only supportive and cannot be used as a substitute for histopathologic diagnosis.…”

Section: Imaging Studiesmentioning

confidence: 91%

Alveolar Capillary Dysplasia

Bishop

Stankiewicz

Steinhorn

2011

Am J Respir Crit Care Med

210

220

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“…In addition, poor NO responsiveness may be related to myocardial dysfunction or systemic hypotension, severe pulmonary vascular structural disease and unsuspected or missed anatomic cardiovascular lesions (such as total anomalous pulmonary venous return, coarctation of the aorta, alveolar capillary dysplasia and others). Prolonged need for iNO therapy without resolution of disease should lead to a more extensive evaluation to determine whether previously unsuspected anatomic lung or cardiovascular disease is present (e.g., pulmonary venous stenosis, alveolar capillary dysplasia, severe lung hypoplasia or others) [73,74] .…”

Section: Therapeutic Strategiesmentioning

confidence: 99%

Recent Advances in the Pathogenesis and Treatment of Persistent Pulmonary Hypertension of the Newborn

2007

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Persistent pulmonary hypertension of the newborn (PPHN) is a clinical syndrome characterized by failure of the lung circulation to achieve or sustain the normal drop in pulmonary vascular resistance (PVR) at birth. Past laboratory studies identified the important role of nitric oxide (NO)-cGMP signaling in the regulation of the perinatal lung circulation, leading to the development and application of inhaled NO therapy for PPHN. Although inhaled NO therapy has improved the clinical course and outcomes of many infants, pulmonary hypertension can be refractory to inhaled NO, suggesting the need for additional approaches to severe PPHN. To develop novel therapeutic strategies for PPHN, ongoing studies continue to explore basic mechanisms underlying the pathobiology of PPHN in experimental models, including strategies to enhance NO-cGMP signaling. Recent studies have demonstrated that impaired vascular endothelial growth factor (VEGF) signaling may contribute to the pathogenesis of PPHN. Lung VEGF expression is markedly decreased in an experimental model of PPHN in sheep; inhibition of VEGF mimics the structural and functional abnormalities of PPHN, and VEGF treatment improves pulmonary hypertension through upregulation of NO production. Other studies have shown that enhanced NO-cGMP activity through the use of cGMP-specific phosphodiesterase inhibitors (sildenafil), soluble guanylate cyclase activators (BAY 41-2272), superoxide scavengers (superoxide dismutase), and rho-kinase inhibitors (fasudil) can lead to potent and sustained pulmonary vasodilation in experimental PPHN. Overall, these laboratory studies suggest novel pharmacologic strategies for the treatment of refractory PPHN.

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Alveolar Capillary Dysplasia: Diagnostic Potential for Cardiac Catheterization

Cited by 18 publications

References 15 publications

Histologic Evidence of Intrapulmonary Anastomoses by Three-Dimensional Reconstruction in Severe Bronchopulmonary Dysplasia

Histologic Evidence of Intrapulmonary Anastomoses by Three-Dimensional Reconstruction in Severe Bronchopulmonary Dysplasia

Alveolar Capillary Dysplasia

Recent Advances in the Pathogenesis and Treatment of Persistent Pulmonary Hypertension of the Newborn

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