Thursday, 28 November 2013

Pulmonary vascular disease – Pulmonary veno-occlusive disease – The Treatments

Pulmonary vascular disease is defined as a condition of blood flow to the lung’s artery is blocked suddenly due to a blood clot somewhere in the body, including pulmonary embolism, chronic thromboembolic disease, pulmonary arterial hypertension, pulmonary veno-occlusive disease, pulmonary arteriovenous malformations, pulmonary edema, etc.
Pulmonary veno-occlusive disease
Pulmonary veno-occlusive disease (PVOD) is an extremely rare form of pulmonary hypertension, affecting mostly in children and young adults as a result of  a progressive obstruction of small pulmonary veins that leads to elevation in pulmonary vascular resistance and right ventricular failure.
VI. Treatments In conventional medicine perspective
A. Non surgical therapy
1. Oxygen therapy 
Total correction was performed in a case of complete transposition of the great arteries (TGA) with severe pulmonary vascular obstructive disease (PVOD). Although severe pulmonary hypertension remained after surgery, oxygenation was continued for 15 months, which included a shift to at-home oxygen inhalation therapy (HOT). Cardiac catheterization 15 months after surgery demonstrated that pulmonary hypertension was greatly improved, According to the study by the Nagoya University Graduate School of Medicine(49).
2. Warfarin therapy
In PVOD, one could suggest that caution should be taken in the application of anticoagulation, because of the frequent association with occult alveolar hemorrhage. However, we have reported the same proportion of hemoptysis in idiopathic PAH and PVOD, even with the use of anticoagulation, according to the study by Alice Huertas, MD; Barbara Girerd, MD; Peter Dorfmuller, MD, PhD; Dermot O’Callaghan, MD; Marc Humbert, MD, PhD; David Montani, MD, PhD(50).
3.  Immunosuppressive agents
In the study of 14 subjects with PAH, including seven patients with the idiopathic form and seven patients with atrial septal defects, but no other congenital heart abnormalities, indicated that chronic administration of sildenafil improves the physical capacity of PAH patients and may be beneficial in selected cases of veno-occlusive disease(51).
B. Lung Transplantation
According to the study by the, Single living-donor lobar lung transplantation is a unique method of treatment, mostly for small children, and size matching is the most important factor to conduct single living-donor lobar lung transplantation safely. We report a successful single living-donor lobar lung transplantation for a 6-year-old girl with pulmonary venoocclusive disease who received the graft from her mother. Preoperatively, the recipient was intubated under deep sedation because of repeated episodes of pulmonary edema due to rapidly deteriorating pulmonary venoocclusive disease(52).
As the prognosis in PVOD patients is worse than in idiopathic PAH, and due to limited medical therapy, lung transplantation should be considered and discussed early in the course of PVOD or suspected PVOD (HRCT findings, worsening symptoms on treatment or refractoriness to medical therapy, development of worsening hypoxemia or chronic heart failure). In these patients, PAH-specific therapies may serve as a bridge to lung transplantation(50). Other study indicated that patients with PVOD present with symptoms of right-sided heart failure. Radiologic examination shows prominent pulmonary arteries with Kerley B lines, pleural effusion, and mediastinal adenopathy. The definite diagnosis is made by histologic examination. Eccentric intimal fibrosis and recanalized thrombi in pulmonary veins and venules, arterialized veins, alveolar edema, and medial hypertrophy of arteries are seen on lung biopsy. No effective treatment is available; lung transplantation has been tried. The prognosis associated with PVOD is poor(53).
General recommendations include limiting of physical activity to tolerance and avoidance of concomitant medications that can potentially aggravate pulmonary hypertension (such as β-adrenergic receptor blockers) or interfere with the metabolism of vitamin K antagonist anticoagulation therapy. Vaccinations to prevent pneumococcal pneumonia and influenza are advised. Given a possible etiological link between tobacco exposure and PVOD, smoking cessation advice and appropriate pharmacotherapy is particularly important and should routinely be offered to cigarette smokers. Diuretics offer symptomatic benefit in those with right ventricular volume overload that is not controlled by dietary measures alone. The effect of diuretics on mortality in PVOD has not been systemically examined(50).

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