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By Dr Teoh Chee Kiang , Consultant Cardiologist

“ The constriction slows blood flow through the lungs , causing the blood pressure in the lungs arteries to rise .

However , only very few may know about the rare and life-threatening pulmonary hypertension disease which affects the arteries of the lung and the right side of the heart .

Unlike systemic blood pressure , which represents the blood pressure circulating in one ’ s body , pulmonary blood pressure indicates the force of the blood flow in the lungs .

According to Institut Jantung Negara ( IJN ) consultant cardiologist Dr Teoh Chee Kiang , pulmonary hypertension is caused by constricted blood vessels in the lung .

“ The constriction slows blood flow through the lungs , causing the blood pressure in the lungs arteries to rise . “ Therefore , the heart must work harder to pump the blood through the lungs , which will make the heart muscle weak and eventually lead to heart failure .”

Citing data from Europe , Dr Teoh said the prevalence of the condition ranges between 10 and 52 cases per million .

“ Based on that figure , we can estimate that there are about 300 to 1,500 patients in Malaysia living with the condition .

IJN is currently taking care of over 600 patients with pulmonary arterial hypertension . The disease is mostly common among young women aged between 20 and 40 .

Symptoms According to Dr Teoh , the signs and symptoms of pulmonary hypertension develop gradually . He noted that the common sign of the condition includes shortness of breath or exertion .

“ Those living with the condition may often experience shortness of breath while carrying out normal routine activities such as household chore and mild exercise .” Some patients may also have a chronic cough . However , those with a more severe condition will have leg and tummy swelling due to water retention .

“ Some of them can even have a dizzy spell and loss of consciousness when they exert themselves too much ,” said Dr Teoh .

Causes On top of its rare nature , Dr Teoh said pulmonary hypertension is a complex disease that is divided into five groups .

Elaborating the causes of each group , he said Group 1 can be categorised as non-cause ( idiopathic ) drug induced and heritable pulmonary hypertension .

“ Sometimes doctors can ’ t find a reason for high blood pressure in the lungs . Hence , in that case , the condition is called idiopathic pulmonary hypertension .

“ A genetic mutation passed down through families may also play a role in why some people get it .”

Dr Teoh said that some of the cases in Group 1 – which has higher prevalence – can also be due to drug-induced pulmonary hypertension caused by a slimming drug called Oraxin .

“ There is also associated pulmonary arterial hypertension which can be due to five other causes , such as a hole in the heart , connective tissue disease , HIV infection , Schistosomiasis ( also known as snail fever and bilharzia ) as well as liver disease .”

Group 2 cases are due to left-side heart disease while Group 3 is caused by lung disease .

Additionally , Group 4 cases are due to blood clots inside the lung vessel .

Dr Teoh said Group 5 includes patients that have pulmonary hypertension caused by other health conditions such as kidney disease , thyroid disease , blood disorder and a rare condition called Sarcoidosis .

Diagnosis Pulmonary hypertension is a progressive condition that develops slowly without early symptoms .

According to Dr Teoh , the condition is very difficult to diagnose as it comes with non-specific early signs .

“ Usually patients living with early-stage pulmonary hypertension will take about one to two years to be diagnosed .

“ The problem is that when it takes a long time to diagnose it , the disease has already progressed to an advanced stage .”

When diagnosed late , Dr Teoh said the patient ’ s heart will already be weaker , which can make the treatment more challenging .

The diagnosis can be done through various health screenings , including X-ray , electrocardiogram and echocardiogram .

“ Of all three , an echocardiogram is the most effective screening option to determine whether we should proceed with further investigations or not .

“ If the echocardiogram tells us that the patient has intermediate or high probability , we will continue with additional checks to diagnose the disease and its group ,” he said .

The additional work up include , blood test , CT-scan and right heart catheterisation – a procedure where a catheter is passed into a vein in the patient ’ s neck or groin to measure the lung pressure .

12 APRIL 2021 GlobalHealthAsiaPacific . com