Case History : A Young Man with PPH....

Ali  Johar, [ not his real name of course ],27 year old junior technician working with a port authority in Pasir Gudang , Johore, walked into my clinic two days ago complaining of 'progressive shortness of breath' over two months duration. He told me he needed to stop twice for breath walking from the underground car park to the hospital lobby. Coming from a 'vital' 26 year old , I was immediately all 'ears'.
Examination was remarkably normal apart from a 'pursed' lip while breathing. He appeared fairly breathless just sitting there on the couch. This is not good. Not good at all. No overt evidence of heart failure.

ECG and Echocardiogram done at the same visit 'shocked' me. Routine chest x-ray was normal except for  an over-prominent pulmonary arterial shadow. That clinched the instant diagnosis of Primary Pulmonary Hypertension, a  very rare ailment.

CT  of the chest  and doppler echo studies of the leg veins done in the same evening, more or less confirmed the diagnosis and exclude the possibility of another cause for his 'breathing difficuly, a  relatively common diagnosis in camparision with PPH  ie ' chronic pulmonary embolism... a condition where you get continued, chronic,  'plugging of the lung circulation by minute blood clots ,over time, coming from the deep veins in the legs and pelvic region.'

His ECG showed obvious 'right heart strain' and in the echo, the right heart [ right ventrical and right atrium ] are both enlarged! His PA systolic pressure [ pressure in the lung circulation, normally less than 20 mmHg ]on echo assessment is 115 mmHg , as high as his systemic pressure on the left. This is bad, prognostically- speaking.


PPH video:-

What is my specific plan for this unfortunate young man?

I  am referring him to my  former student and junior colleague in IJN who 'subspecialise' on this disease and is currently the  director of  their 'heart and lung' transplantation programme. Six months to two years down the lane, my gut-feeling is that, this young man would need a heart and lung transplant, judging by his markedly raised PA systolic pressure now.  Whether he would get one is another matter I would not want to ponder about. Waiting list  is long 'plus plus', and hearts and lung from 'donors' very in very short supply. Only 'accident cases' in  'brain dead'  circumstances qualifies.  Even then, family consent is difficult to get by. This is the real world...

Even if he gets one,  one year down the 'lane', there is the incessant  problem of host versus graft disease; graft versus host disease; and myriads of other issues; and last but by no mean the least,  is cost.

In the interim period, IV prostacycline infusion initially,  and the new oral ones recently introduced may tide him over. Very expensive treatment , a couple thousand RM a  month! Oral viagra or cialis , which help lower the pulmonary circulation pressure have been helpful in some, giving a new hope of better symptom control in many at less cost.

How do one tell  all these to a previously bubbly 26 year old, younger even than even my sons and daughter;recently married young chap; with a  young innocent wife and a six month baby in tow?!  Just three months ago he  was still playing 'futsal' with his work colleagues in JB......
That in practical terms, his life will be  drastically 'truncated'.
.
I am reminded of an old professor of medicine at Massachusett General, Prof  Sir William Osler, doyen of American medical fraternity at the turn of the previous century, who used to say: " The science of medicine is long and arduous, the art is even longer.."
How very true. I am still learning......

Inna lillah hiwainna ilaihirojiun.
From Him we come , to Him we return.