Pulmonary Hypertension

Causes of Pulmonary Hypertension ,Physical Examination and Symptoms of Pulmonary Hypertension with Pulmonary Hypertension Treatment Pulmonary hypertension means an increase in pulmonary artery pressure. Pulmonary hypertension (PH) is characterized by elevated pulmonary arterial pressure and secondary right ventricular failure. Elevated blood pressures in the pulmonary arteries (above 25-30 mm Hg). Normal pulmonary artery peak systolic pressure is 15-30 mm Hg.

The cause of pulmonary hypertension may be car­diac, pulmonary, or vascular. Some degree of pulmo­nary hypertension also occurs due to ageing.

• Pulmonary hypertension can occur as a back pres­sure from the left side of heart i.e. left atrium, pul­monary veins and left ventricle.
• Pulmonary hypertension also occurs due to hypoxia, obstruction, vasoconstrictor stimuli, degenerative changes in the pulmonary vascular bed.
• Pulmonary hypertension is the most common cause of cor pulmpnale,
• Persistent pulmonary hypertension can lead to right heart failure.

 

Pulmonary Hypertension CLASSIFICATION —

 

• The Pulmonary Hypertension PH was previously classified as either idiopathic pulmonary arterial hypertension (IPAH, formerly called primary pulmonary hypertension) or secondary Pulmonary Hypertension PH.

 

Primary pulmonary hypertension

 

• Pulmonary arterial hypertension (PAH)
• Pulmonary Hypertension is a rare familial illness in which small pulmonary arteries become blocked as a result of abnormalities in the structure of blood vessels in the lung.
• Drug- and toxin-induced PAH is also considered

 

Secondary pulmonary hypertension

 

• hematologic disorders (eg, myeloproliferative disorders)
• Pulmonary Hypertension due to elevation in pulmonary artery pressure as a result of left ventricular failure, blood clots in the pulmonary arteries, or chronic lung diseases.
• metabolic disorders (eg, glycogen storage disease)

Causes of Pulmonary Hypertension

   

• Obstructive sleep apnea (OSA) — A review of eight studies estimated that the prevalence of mild PH is 15 to 20 percent among patients with OSA
• Interstitial pulmonary fibrosis
• Mitral valve disease
• VSD, PDA, ASD
• Systemic sclerosis (SSc, formerly called scleroderma)
• Chronic obstructive lung disease ~ Chronic parenchymal disease -” Chronic hypoxia
• Pulmonary thromboembolism – Venoocclusive disease
• Approximately 30 percent of children who do not have their congenital heart disease repaired will develop PAH
• HIV infection
• Anorexigens – aminorex, fenfluramine ~ Pulmonary capillary hemangiomatosis ~ LV diastolic dysfunction
• CREST syndrome
• Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly Telangietasia
• Scleroderma
• SLE
• Sjogren’s syndrome
• Dermatomyositis
• Polymyositis
• Rheumatoid arthritis ~ Sleep disorders
• Chronic hypoventilation
• Thoracovertebral deformities
• Neuromuscular diseases
• Diaphragmatic paralysis
• Sickle cell disease
• Sarcoidosis
• Portal hypertension
• Schistosomiasis
• Primary pulmonary hypertension (PPH) -. rare
• cause.

 

Symptoms of Pulmonary Hypertension

   

• Exertional dyspnoea .y Fatigue
• Angina pectoris
• Syncope
• Peripheral edema
• Chest pain.

 

Pulmonary Hypertension Physical Examination

   

• Increased NP
• RV Lift or Parasternal heave ,
• P₂ loud
• RVS₄
• Later there is peripheral cyanosis ,
• Edema
• Clubbing
• Ejection systolic murmur in the left parasternal area
• Pulmonary ejection sound ..y RV 53
• Graham Steell murmur – Early diastolic murmur in left PSA
• TR.

Chest X-ray of Pulmonary Hypertension

   

• · Enlarged pulmonary arteries
• · The findings pertaining to cause of PH.

Pulmonary Hypertension ECG

   

• RAD, RVH, RAE, P pulmonale
• M – mitrale.

Pulmonary Hypertension Echocardiogram

   

• · RVH
• · RAE
• · TR
• · Increased RVSP(Right ventricular systolic pres­sure)
• · Increased PASP (Pulmonary artery systolic pres­sure)
• Pulmonary function tests Suggestive of :
• Obstructive airway disease Restrictive lung disease.
• Oximetry for
• • Hypoxaemia.
• Perfusion lung scan may show Pulmonary thromboembolism.

 

Other tests

   

• · Antinuclear antibody
• · HIV
• · Thyroid function tests (for association with PPH ie Primary Pulmonary Hypertension).

 

Cardiac catheterization for

 

• · Pulmonary artery pressure
• · Cardiac Output
• · Cardiac shunts
• · LVEDP ( LV end-diastolic pressure)
• The reversibility of pulmonary hypertension is tested with inhaled nitric oxide, IV adenosine, which reduce pulmonary artery pressure acutely.

 

Stages of pulmonary arterial hypertension

   

• · Increase of pulmonary vascular pressures
• · Increased pulmonary vascular resistance
• · Pulmonary vasospasm
• · Medial hypertrophy
• · Eccenteric and concentric intimal fibrosis
• · Thrombi in pulmonary vasculature
• · Degeneration and necrosis of blood vessels.

 

Pulmonary Hypertension Treatment

   

• Avoid exertion Digitalis Diuretics
• Oxygen supplementation
• Anticoagulant therapy – Warfarin should be given to all patients of PPH (Primary Pulmonary Hypertension)
• Calcium channel blockers – Nifedipine, Amlodipine
• Prostacyclin – Epoprostenol
• Endothelin receptor antagonists – Bosentan Sildenafil
• Lung transplantation.