pulmonary hypertension
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Clinical Classification of Pulmonary Hypertension
Primary pulmonary hypertension
Category 1: PAH
- idiopathic primary pulmonary hypertension (IPPH)
- Familial
- Pulmonary hypertension associated with:
- Collagen vascular disease
- Congenital systemic-to-pulmonary shunts
- Portal hypertension
- Drugs/toxins
- HIV infection
- Other (Gaucher’s, hereditary hemorrhagic telangiectasia, hemoglobinopathies, splenectomy)
- Associated with significant venous or capillary involvement
- Pulmonary veno-occlusive disease
- Pulmonary capillary hemangiomatosis
- Persistent pulmonary hypertension of the newborn
Secondary pulmonary hypertension (Non–Category 1)
Category 2: Pulmonary venous hypertension
Category 3: Pulmonary hypertension associated with disorders of the respiratory system and/or hypoxemia
Category 4: Pulmonary hypertension due to chronic thrombotic and/or embolic disease
Category 5: Pulmonary hypertension due to miscellaneous disorders directly affecting the pulmonary vasculature
- sarcoidosis
- histocytosis X
- lymphangiomatosis
- compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis)
Types
acute pulmonary hypertension
chronic pulmonary hypertension
Grading system
Heath-Edwards system
Etiology
primary pulmonary vascular diseases
- primary pulmonary hypertension (PPH)
- persistent pulmonary hypertension of the newborn (PPHN) or persistent fetal circulation (PFC)
secondary pulmonary hypertension
- meconium aspiration
- pulmonary parenchymal diseases
- bacterial pneumonia
- postviral obliterative bronchiolitis
- bronchopulmonary dysplasia
- acute respiratory distress syndrome (ARDS)
- pulmonary interstitial fibrosis
- airways diseases
- persistent upper airway obstruction
- chronic airway obstruction (asthma)
- chronic lower airway obstruction (cystic fibrosis)
- endstage pulmonary disease
- premature closure of ductus arteriosus
- premature closure of foramen ovale
- alveolar capillary dysplasia with misalignement of pulmonary vessels
- pulmonary veno-occlusive disease
- invasive pulmonary capillary haemangiomatosis
- pulmonary embolic disease
- thromboembolism
- non-thrombotic embolism
- congenital and acquired heart diseases
- with pulmonary venous hypertension
- obstruction to left ventricular outflow
- aortic atresia
- aortic stenosis
- hypoplastic left heart
- aortic coarctation with VSD
- interrupted aortic arch
- obstruction to left ventricular outflow
- with pulmonary venous hypertension
- obstruction to pulmonary venous return
- critical mitral stenosis
- cor triatrium
- pulmonary venous stenosis
- intradiaphragmatic TAPVR
- obstruction to pulmonary venous return
- decreased left ventricular contractility
- transient left ventricular dysfuntion
- endocardial fibroelastosis
- glycogen storage disease type II (GSD2) (Pompe disease)
- decreased left ventricular contractility
- with increased pulmonary blood flow
- transposition of great arteries
- supradiaphragmatic TAPVR
- endocardial cushion defect
- arteriovenous malformations
- with increased pulmonary blood flow
- with isolated VSD
- decreased pulmonary vascular bed
- pulmonary hypoplasia
- primary pulmonary hypoplasia
- secondary pulmonary hypoplasia
- congenital diaphragmatic hernia
- oligohydramnios sequence
- asphyxiating thoracic dystrophy (Jeune syndrome)
- fetal pleural effusions
- generalized and local vascular disorders
- portocaval shunt
- collagen vascular diseases (dysimmune diseases)
- intravenous drug abuse
- vascular toxins
- drugs
- mechanical disorders
- chest wall restriction (kyphoscoliosis)
- muscle weakness (myasthenia gravis)
- central nervous system disorders
- acquired (head injury, ence^phalitis)
- congenital (Arnold-Chiari malformations)
- ventilatory control diorders
- blood hyperviscosity
- polycythemia
- fetal-maternal transfusion
- fetal-fetal transfusion
- sickle-cell disease
- high-altitude residence
- antiphospholipid antibody syndrome
- sleep disordered breathing due to obstructive sleep apnea
- neuromuscular disease
- portocaval shunt (#12447666#, #6886359#)
- portal hypertension (#159649#)
- cirrhosis of the liver with portacaval shunts (#5634734#)
Associations
portal hypertension (#1409142#)
Grading system
Heath-Edwards grading system of pulmonary hypertension
See also
pulmonary plexogenic arteriopathy
References
Tulloh RM. Congenital heart disease in relation to pulmonary hypertension in paediatric practice. Paediatr Respir Rev. 2005 Sep;6(3):174-80. PMID: #16153566#
Bush A. Pulmonary hypertensive diseases. Paediatr Respir Rev. 2000 Dec;1(4):361-7. PMID: #16263465#
Alt B, Shikes RH. Pulmonary hypertension in congenital heart disease: irreversible vascular changes in young infants. Pediatr Pathol. 1983 Oct-Dec;1(4):423-34. PMID: #6687292#
Pathology references
Wagenvoort CA. Grading of pulmonary vascular lesions—a reappraisal. Histopathology. 1981 Nov;5(6):595-8. PMID: #7319479#
Yi ES, Kim H, Ahn H, Strother J, Morris T, Masliah E, Hansen LA, Park K, Friedman PJ. Distribution of obstructive intimal lesions and their cellular phenotypes in chronic pulmonary hypertension: a morphometric and immunohistochemical study. Am J Respir Crit Care Med 2000;162:1577–1586
Smith P, Heath D, Yacoub M, Madden B, Caslin A, Gosney J. The ultrastructure of plexogenic pulmonary arteriopathy. J Pathol 1990;160:111–121.
Heath D, Smith P, Gosney J, Mulcahy D, Fox K, Yacoub M, Harris P. The pathology of the early and late stages of primary pulmonary hypertension. Br Heart J 1987;58:204–213.
Wagenvoort CA. Lung biopsies and pulmonary vascular disease. In: Weir EK, Reeves JT, editors. Pulmonary hypertension. Mount Kisco, NY: Futura Publishing Co; 1984. pp. 393–438.
Reid LM. Structure and function in pulmonary hypertension: new perceptions. Chest 1986;89:279–288.