Lung and heart-lung transplant practice patterns in pulmonary hypertension centers

Pielsticker, Elizabeth; Martínez, Fernando J.; Rubenfire, Melvyn

doi:10.1016/s1053-2498(01)00348-5

Cited by 57 publications

(25 citation statements)

References 19 publications

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“…However, lung transplantation can only be considered in a minority of patients with end-stage pulmonary diseases and long-term benefits remain disappointing, with ,50% survival at 5 yrs [89,90]. Mono-pulmonary transplantation has good long-term results [91,92] but most centres currently prefer bi-pulmonary or cardiopulmonary transplantation, which have fewer post-operative complications [90,93]. Because of the worse prognosis of PVOD patients, it may be necessary to discuss lung transplantation early in the course of PVOD.…”

Section: Lung Transplantationmentioning

confidence: 99%

Pulmonary veno-occlusive disease

Montani¹,

Price²,

Dorfmüller³

et al. 2009

European Respiratory Journal

269

250

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Pulmonary veno-occlusive disease (PVOD) is currently classified as a subgroup of pulmonary arterial hypertension (PAH) and accounts for 5-10% of cases initially considered to be idiopathic PAH. PVOD has been described as idiopathic or complicating other conditions, including connective tissue diseases, HIV infection, bone marrow transplantation, sarcoidosis and pulmonary Langerhans cell granulomatosis. PVOD shares broadly similar clinical presentation, genetic background and haemodynamic characteristics with PAH. Compared to PAH, PVOD is characterised by a higher male/female ratio, higher tobacco exposure, lower arterial oxygen tension at rest, lower diffusing capacity of the lung for carbon monoxide, and lower oxygen saturation nadir during the 6-min walk test. High-resolution computed tomography (HRCT) of the chest can be suggestive of PVOD in the presence of centrilobular ground-glass opacities, septal lines and lymph node enlargement. Similarly, occult alveolar haemorrhage is associated with PVOD. A noninvasive diagnostic approach using HRCT of the chest, arterial blood gases, pulmonary function tests and bronchoalveolar lavage could be helpful for the detection of PVOD patients and in avoiding high-risk surgical lung biopsy for histological confirmation. PVOD is characterised by a poor prognosis and the possibility of developing severe pulmonary oedema with specific PAH therapy. Lung transplantation is the treatment of choice. Cautious use of specific PAH therapy can, however, be helpful in some patients.KEYWORDS: Alveolar haemorrhage, BMPR2, computed tomography, diffusing capacity of the lung for carbon monoxide, pulmonary arterial hypertension, pulmonary veno-occlusive disease P ulmonary arterial hypertension (PAH) is a severe condition characterised by elevated pulmonary artery pressure leading to right heart failure and death [1,2]. Pulmonary veno-occlusive disease (PVOD) is classified as a subgroup of PAH and accounts for 5-10% of histological forms of cases initially considered to be idiopathic PAH. Even though the first welldocumented case of PVOD was described .70 yrs ago, the characteristics and pathophysiology of this disease remain poorly understood [3,4]. While pulmonary vascular pathology of idiopathic or familial PAH is characterised by a major remodelling of small pre-capillary pulmonary arteries with typical plexiform and/or thrombotic lesions, PVOD preferentially affects the post-capillary venous pulmonary vessels [5,6]. Despite this anatomical histological difference, PVOD has a very similar clinical presentation to PAH but is characterised by a worse prognosis and the possibility that severe pulmonary oedema can develop with specific PAH therapy, justifying the importance of diagnosing this disease. A definitive diagnosis of PVOD requires histological analysis of a lung sample [7,8]; however, surgical lung biopsy is a high-risk procedure in these patients and the development of a less invasive diagnostic approach would be preferable [9][10][11]. The present manuscript will summa...

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Section: Lung Transplantationmentioning

confidence: 99%

Pulmonary veno-occlusive disease

Montani¹,

Price²,

Dorfmüller³

et al. 2009

European Respiratory Journal

269

250

View full text Add to dashboard Cite

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“…Intrinsic LV myocardial abnormalities might help to explain late recovery of LV filling after single-lung transplantation in patients with PH, even if LV geometry and RV function are immediately restored [50]. They might explain as well, why combined heartlung transplantation is favored for PH patients when severe impairment of LV function is present, since LV function may not sufficiently recover after transplantation of the lungs alone [39].…”

Section: Introductionmentioning

confidence: 99%

Time course and mechanisms of left ventricular systolic and diastolic dysfunction in monocrotaline-induced pulmonary hypertension

et al. 2009

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Although pulmonary hypertension (PH) selectively overloads the right ventricle (RV), neuroendocrine activation and intrinsic myocardial dysfunction have been described in the left ventricle (LV). In order to establish the timing of LV dysfunction development in PH and to clarify underlying molecular changes, Wistar rats were studied 4 and 6 weeks after subcutaneous injection of monocrotaline (MCT) 60 mg/kg (MCT-4, n = 11; MCT-6, n = 11) or vehicle (Ctrl-4, n = 11; Ctrl-6, n = 11). Acute single beat stepwise increases of systolic pressure were performed from baseline to isovolumetric (LVPiso). This hemodynamic stress was used to detect early changes in LV performance. Neurohumoral activation was evaluated by measuring angiotensin-converting enzyme (ACE) and endothelin-1 (ET-1) LV mRNA levels. Cardiomyocyte apoptosis was evaluated by TUNEL assay. Extracellular matrix composition was evaluated by tenascin-C mRNA levels and interstitial collagen content. Myosin heavy chain (MHC) composition of the LV was studied by protein quantification. MCT treatment increased RV pressures and RV/LV weight ratio, without changing LV end-diastolic pressures or dimensions. Baseline LV dysfunction were present only in MCT-6 rats. Afterload elevations prolonged s and upward-shifted end-diastolic pressure dimension relations in MCT-4 and even more in MCT-6. MHC-isoform switch, ACE upregulation and cardiomyocyte apoptosis were present in both MCT groups. Rats with severe PH develop LV dysfunction associated with ET-1 and tenascin-C overexpression. Diastolic dysfunction, however, could be elicited at earlier stages in response to hemodynamic stress, when only LV molecular changes, such as MHC isoform switch, ACE upregulation, and myocardial apoptosis were present.

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“…Survival in patients with PAH having lung transplantation is approximately 66% to 75% at 1 year (94). Most centers prefer bilateral lung transplantation for patients with PAH (95).…”

Section: Interventional and Surgical Therapiesmentioning

confidence: 99%

Evaluation and Management of the Patient with Pulmonary Arterial Hypertension

Rubin

Badesch²

2005

Ann Intern Med

126

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Increased pressure in the pulmonary circulation, or pulmonary hypertension, is a common disorder that may complicate various cardiopulmonary conditions, including severe obstructive airways disease and left ventricular dysfunction. An increase in pulmonary arterial pressure that is not due to coexistent cardiopulmonary disease, known as pulmonary arterial hypertension, may occur in the absence of a demonstrable cause (idiopathic or familial); as a complication of systemic conditions, such as connective tissue disease, HIV infection, or chronic liver disease; or as a result of the use of fenfluramine anorexigens, amphetamines, or cocaine. The development of disease-specific therapies for pulmonary arterial hypertension over the past decade underscores the importance of diagnosing pulmonary hypertension early in the course of the condition and implementing a treatment strategy that is based on the condition's cause and severity. In this review, the authors present approaches to the diagnosis and management of pulmonary arterial hypertension, using a hypothetical case to highlight the key management points. U ntil recently, management of pulmonary arterial hypertension (PAH) was generally ineffective in alleviating symptoms or improving survival. However, the past decade has witnessed remarkable advances in our understanding of the pathogenesis of PAH, advances that have led to the development of disease-specific treatments. Despite these achievements, PAH remains a challenging condition to diagnose and manage. This article reviews recent developments in the diagnosis and management of PAH in the context of a typical case, illustrating the importance of collaboration between the internist and the specialist in patient care.

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Lung and heart-lung transplant practice patterns in pulmonary hypertension centers

Cited by 57 publications

References 19 publications

Pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease

Time course and mechanisms of left ventricular systolic and diastolic dysfunction in monocrotaline-induced pulmonary hypertension

Evaluation and Management of the Patient with Pulmonary Arterial Hypertension

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