Tuesdays with Chandra: Pulmonary vein elopes

The hospital staff was pushing the stretcher and there I was lying in a loose fitting hospital garb. I saw two of my friends and then my parents following me. Sometimes I would lose sight of them in the crowd, and then I would slowly lift my head to get a glance. I had no clue where my brother vanished, he was not someone small, but nevertheless he was missing. An inch long vibuthi adorned the center of my forehead with a tiny mark of kumkum could not hide the anxiety of my face. And the only ones who were cool and calm were the hospital staff who pushed the stretcher.

I had to leave everything and everyone behind and it all started with shaving my chest in the morning and soaking every pore with Betadine. The razor could clean everything on my chest and the solution could only clean my outer skin, but the memories of loved ones and family was intact. For the last time I said bye to my parents and it felt like my first day ever in school, my eyes brimming with tears and over-sized heart filled with memories. I really wanted to hold their hands, just like a new-born does out of insecurity. When I was a new born my five fingers would have curled up around their one finger, but today they were faraway though I could clasp all their fingers with mine.

I was whisked into the theatre area and I was made to wait in the runway for my chance. For one last time I begged the hospital staff to show me my parents and friends. I looked at the glass opening in the door and I couldn’t see any of my dear ones peeping through. I told him I don’t mind getting of the stretcher and walking out to see them for one last time. I didn’t have any money on me to bribe him and all I could do was convince him with my tone and tears. I know my request sounded hideous, but the guy understood my state of mind and obliged to call them in. Two of my friends came in, but my parents had gone down to the waiting area. I held their hands, not sure whose hands were colder, mine or theirs. When I woke up it was 3.30 pm in the afternoon and all this was happening eight weeks before the surgery.

That morning of April 18 after the round of extended tests, I sat with my doctor friend at the lunch table to understand the real issue in my heart. He took the paper napkin started to sketch the anatomy of heart and my anomaly. Should I call it storyboarding? None of the medical terms like “Partial anomalous pulmonary venous return” (PAPVR) made any sense to me. I was still in a state of disbelief sitting in front of him and none of what he said entered my ears and registered in my cortex.

In simple Bollywood parlance, of the four Pulmonary veins that carries oxygenated blood from the lungs to the left side of the heart, one of them had grown tall and eloped and joined the bridging vein aka innominate vein (carries deoxygenated blood) and drains into the right atrium. And to me this news sounded like an inter-caste marriage, a marriage between the oxygenated vein and deoxygenated vein and my heart was harboring the matrimonial secret for 35 years and unwilling to part with it. Was it really a marriage or an affair? It could be whatever, but doctors were now planning to open my chest, separate the vein and put it back in the right chamber. And it is going to be a tough fight, and there would be surgical weapons and some blood shed. Will the left side of my heart accept the new tenant? We have to wait and watch!

My first two questions to the doctor were very basic, how could it happen to me and how come it went undetected for 35 years? I felt fit all through my life, there was breathless only when I pushed my lungs to its limits and that is common among athletes. I have lifted weights for the past 11 years, practiced yoga regularly, actively involved in sports of every kind and even ascended 4 of the 6 sacred peaks in Himalayas. And his answers were plain and simple. PAPVR is congenital; it is asymptomatic and usually revealed on an x-ray only when dilatation of heart happens or later on in life (late 50s) when people are diagnosed with arrhythmias.

What is PAPVR?

Partial Anomalous pulmonary venous return (PAPVR) is a rare heart defect that occurs when the pulmonary veins fail to form normally while the baby is in the mother’s womb. It comprises <1% of all congenital heart defects. The cause of the problem is not known.

In the normal heart, there are four pulmonary veins that bring red blood back from the lungs to the heart’s left upper chamber (the left atrium). Two of the veins bring red blood from the right lung and two bring red blood from the left lung. In a baby with anomalous pulmonary venous return, one or more of the pulmonary veins returns to the right atrium instead of the left atrium.

As a result of this draining, there was volume overload on the right side of the heart (this explained the dilatation) and the lung (luckily, pulmonary hypertension had not set in) was purifying the same blood again. And my body managed to grow this big with a little less pure blood.

With every spoon of rice going down my gullet, I had a question to ask, I know it sounds like a reflux. To my friend it reminded him of his a viva-voce sessions in the medical school.

So what would happen if we leave the condition untreated?

The right side heart muscles will be irreversibly stretched and damaged; pulmonary hypertension (high blood pressure in the arteries of lungs) will overwork the right side of the heart and eventually lead to heart failure. And it can also give rise to atrial fibrillation (abnormal cardiac rhythm in the right side of the heart).

Surgery was imminent and the eloped vein had to be brought back home to save my life. Bringing home may sound simple, but the procedure involves breaking the sternum or moving around the ribs. And I would have mark(s) of a warrior at the end of the surgery.

How soon should the surgery be performed?

Sooner the better was the answer from the medical fraternity. I had no cholesterol, pressure and diabetes and that significantly decreased the risks in the surgery and recovery time post-surgery. Leaving the right side muscles of the heart dilated for a long time may cause an irreversible damage (stretched beyond Young’s modulus).

The bus ride back home that afternoon was scarier than the afternoon dream last weekend. There was some plumbing and tailoring job to be done in my heart. How do I convey this to my parents? The bus was crowded, but still I felt I like I was the only one there, and route that was punctuated with building every inch seemed deserted. There was a tug-of-war between belief and denial. I was just 35 and my chest bone had to split open? "Why me" was the most difficult question hanging in front of my eyes?

Come back next week to find out how I am coping up with this, and how my near and dear ones are reacting to this news.