Pulmonary Vein Stenosis: Prematurity and Associated Conditions

Drossner, David; Kim, Dennis W.; Maher, Kevin; Mahle, William T.

doi:10.1542/peds.2008-0075

Cited by 160 publications

(157 citation statements)

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“…Pulmonary vein stenosis (PVS) is a rare condition, with a reported prevalence of 1.7 per 100,000 children younger than two years of age (1). It is exceptionally difficult to manage, and is associated with less than a 50% survival rate within five years of diagnosis (1,2).…”

Section: Discussionmentioning

confidence: 99%

“…It is exceptionally difficult to manage, and is associated with less than a 50% survival rate within five years of diagnosis (1,2). PVS has been reported in 0.5% of pediatric autopsy cases (3).…”

Section: Discussionmentioning

confidence: 99%

“…There is a diverse spectrum of disease. The stenosis usually occurs at the anastomosis between the left atrium and the pulmonary vein confluence, or may extend into the individual pulmonary veins (1). A left-sided PVS causes increased back pressure proximal to the stenosis, decreasing left pulmonary artery flow and increasing right pulmonary artery flow.…”

Section: Discussionmentioning

confidence: 99%

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Pulmonary Vein Stenosis: Case Report and Literature Review

Amin

Kwon

Moayedi

et al. 2009

Canadian Respiratory Journal

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BACKGROUND: Pulmonary vein stenosis is a rare cause of pulmonary hypertension, with variable onset and presentation. One or more of the four pulmonary veins can be primarily or secondarily affected. A five-month-old girl presented with respiratory distress, lethargy and cyanosis requiring intubation.METHODS: Echocardiography showed right ventricular dilation, a right ventricular systolic pressure of 97 mmHg, decreased ejection fraction and turbulent flow at the left atrium. Cardiac catheterization revealed stenosis of the left-sided pulmonary veins, for which she underwent a Coles procedure.RESULTS: Postoperatively, there was a transient improvement in the patient’s pulmonary hypertension but she subsequently deteriorated. Her prognosis was considered bleak, and a decision was made with the family to withdraw care.CONCLUSION: Pulmonary vein stenosis is a rare cause of pulmonary hypertension, and is associated with significant morbidity and mortality. Surgical intervention may be of benefit in selected cases. It stands to reason that any treatment will have the best chance of success if completed before the pulmonary hypertension becomes fixed. Early diagnosis depends on a high index of clinical suspicion.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Pulmonary Vein Stenosis: Case Report and Literature Review

Amin

Kwon

Moayedi

et al. 2009

Canadian Respiratory Journal

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“…This occurs in several settings: idiopathic, in prematurely born infants with bronchopulmonary dysplasia, after surgical repair of anomalously connected pulmonary veins (including those not obstructed at time of repair), in patients with congenital heart defects without anomalous pulmonary vein connection, and under other circumstances. 19,20,[23][24][25] It seems unlikely that a single stenotic pulmonary vein can cause significant PH, 26 although manifest obstruction of one or more pulmonary veins in one lung can exist with occult obstruction of small veins in the contralateral lung, and additional obstructed veins can develop over time. 27 ii) Pulmonary veno-occlusive disease.…”

Section: Causes Of Pvhmentioning

confidence: 99%

Pulmonary Hypertension Caused by Pulmonary Venous Hypertension

Kulik

2014

Pulm. circ.

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The effect of pulmonary venous hypertension (PVH) on the pulmonary circulation is extraordinarily variable, ranging from no impact on pulmonary vascular resistance (PVR) to a marked increase. The reasons for this are unknown. Both acutely reversible pulmonary vasoconstriction and pathological remodeling (especially medial hypertrophy and intimal hyperplasia) account for increased PVR when present. The mechanisms involved in vasoconstriction and remodeling are not clearly defined, but increased wall stress, especially in small pulmonary arteries, presumably plays an important role. Myogenic contraction may account for increased vascular tone and also indirectly stimulate remodeling of the vessel wall. Increased wall stress may also directly cause smooth muscle growth, migration, and intimal hyperplasia. Even long-standing and severe pulmonary hypertension (PH) usually abates with elimination of PVH, but PVH-PH is an important clinical problem, especially because PVH due to left ventricular noncompliance lacks definitive therapy. The role of targeted PH therapy in patients with PVH-PH is unclear at this time. Most prospective studies indicate that these medications are not helpful or worse, but there is ample reason to think that a subset of patients with PVH-PH may benefit from phosphodiesterase inhibitors or other agents. A different approach to evaluating possible pharmacologic therapy for PVH-PH may be required to better define its possible utility.Keywords: pulmonary hypertension, pulmonary venous hypertension. As a perfect example of how clinical imperative can drive basic physiological investigation, studies of the effect of pulmonary venous hypertension (PVH) on the lung's circulation were in full swing by the very early 1950s, 1 close on the heels of the development of surgical relief of mitral valve stenosis. Cardiac catheterization of patients with mitral valve disease has characterized the physiology of PVH, and histological investigations demonstrated the microvascular changes provoked by PVH. Just as important, the effect of relief of left atrial (LA) hypertension on the circulation has been extensively reported, usually for patients who have had mitral valve intervention. We have learned that the circulatory alterations provoked by PVH share some characteristics with other forms of pulmonary hypertension (PH), such as the idiopathic variety, PH due to chronic alveolar hypoxia, and PH secondary to congenital cardiac shunting lesions. For example, acute "reactivity" to vasodilators may be observed, and there is overlap in the microarchitectural abnormalities seen. On the other hand, the "natural history" of PVH-PH is very different than that of the idiopathic variety or that seen with shunting defects: in the former (as demonstrated with mitral valve disease), PH is likely to largely or completely resolve if PVH is relieved, even with long-standing and severely increased pulmonary vascular resistance (PVR), 2,3 while in the latter two, PH is more likely to inexorably increase. PVH-PH is ther...

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“…E.g., PH may develop on basis of pulmonary hypoplasia and/or pulmonary vascular development delay and genetic syndromes at BPD or interstitial pulmonary diseases. Patients with BPD may have PH associated with intermittent chronic hypoxia, hypercapnia due to the damaged pulmonary and airway tissue, diastolic dysfunction, pulmonary vein stenosis, congenital heart diseases and pulmonary-to-systemic shunt [4,8,9]. Treatment PH treatment includes: oxygen, diuretics, digoxin (in case circulatory deficiency progresses), anticoagulants, vasodilators (in case vasoreactivity test is positive), endothelin antagonists, prostaglandins and their analogs, phosphodiesterase inhibitors and surgical treatment modes (interatrial shunting, lung transplantation, cardiopulmonary transplantation).…”

Section: Classificationmentioning

confidence: 99%