Idiopathic Pulmonary Arterial Hypertension (IPAH) - Its Presentation, DD, Investigations & Treatment

Idiopathic Pulmonary Arterial Hypertension (IPAH) - Its Presentation, DD, Investigations & Treatment


Idiopathic Pulmonary Arterial Hypertension (IPAH) - Its Presentation, DD, Investigations & Treatment

Watch this informative video on Idiopathic Pulmonary Arterial Hypertension (IPAH), its Presentation, DD, Investigations \u0026 Treatment.

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Content

1.44 -> Hello and welcome to this channel. This video will give you a quick review  
5.52 -> of pulmonology topic, Idiopathic  Pulmonary Arterial Hypertension.
11.92 -> Pulmonary Hypertension (PH) is said to be  
13.68 -> present when a mean pulmonary  artery pressure is > 25 mmHg.  
20.08 -> Idiopathic pulmonary arterial hypertension ,or in  short IPAH is defined as, Pulmonary Hypertension  
25.92 -> with absent lung or left-sided heart disease. It is also termed as pre-capillary pulmonary  
32.4 -> hypertension. And in WHO-classification,  Group-1 pulmonary hypertension. 
38.96 -> It was previously also known as  'Primary Pulmonary Hypertension'. 
44.56 -> Idiopathic pulmonary arterial  hypertension is a rare disease  
48.08 -> with no apparent cause. This rare disorder is  seen primarily in young and middle-aged women.
56.48 -> Untreated, IPAH leads to  right-sided heart failure and death.
63.28 -> Let's talk about its presentation.  The affected patient may present  
67.92 -> with progressive shortness of breath,  malaise, chest pain, or exertional syncope.
75.12 -> Clinical examination may reveal tachycardia, left  parasternal heave due to right ventricular lift,  
80.88 -> loud pulmonary component of second  heart sound (Loud P2), right-sided S3,  
84.88 -> pulmonary and tricuspid regurgitation murmurs,  and in advanced cases, right-sided heart failure,  
91.04 -> evident by jugular venous distension,  peripheral edema, hepatomegaly and ascites.
98.64 -> Investigations that needs to be done include,  electrocardiography (ECG) which shows right  
104.32 -> axis deviation, right bundle branch block,  right ventricular hypertrophy or strain.
111.92 -> Echocardiography will show evidence of  elevated right ventricular systolic pressure  
116.64 -> with or without evidence of  RV dilation or dysfunction. 
121.28 -> Other investigation include  right heart catheterization. 
125.36 -> Differential diagnosis of this condition include  pulmonary venous hypertension due to mitral  
131.2 -> stenosis or left heart failure from any etiology,  sleep apnea and obesity hypoventilation syndromes,  
138.4 -> chronic thromboembolic disease, diffuse  parenchymal lung diseases, chronic obstructive  
144.16 -> pulmonary disease, sympathomimetic drug-induced  pulmonary hypertension, HIV-associated pulmonary  
151.12 -> hypertension, Porto-pulmonary hypertension  in liver cirrhosis with portal hypertension. 
158.24 -> Coming on to treatment options of this condition.  Different therapies are available. Combinations  
164.08 -> of agents are generally more beneficial  than any agent alone. Clinical benefit  
168.48 -> versus side effects must be considered. Calcium channel blockers: About 5% patients  
175.12 -> respond well to calcium channel blockers.  Over the long term, this response is seen  
179.68 -> only in patients who respond to vasodilator  challenge during right heart catheterization. 
185.2 -> Endothelin receptor blockers and  phosphodiesterase 5 inhibitors improve  
189.52 -> symptoms in mild or moderately severe cases. Continuous intravenous prostacyclin analogues  
196 -> are the best studied agents and are  generally initiated in more severe cases. 
201.12 -> Empiric anticoagulation may  confer survival benefit. 
205.52 -> Lung, or heart-lung transplantation should  be considered in appropriate candidates.
222 -> Produced & Uploaded by Last Second Medicine

Source: https://www.youtube.com/watch?v=XWtWJO0wUs8