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Diagnosed with Congenital heart disease, situs solitus, mid muscular VSD bidirectional shunt. Had blood vomit. Serious?

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Cardiologist, Interventional
Practicing since : 1996
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The patient is 38 year old unmarried female diagnosed with Congenital heart disease, situs solitus, mid muscular VSD bidirectional shunt and severe pulmonary hypertension. On 01st april 12, the patient had blood vomit at around 2 am. The patient is feeling weak and have no desire to take food. All these years, no medicines have been prescribed by Doctors consulted. My question is requirement of the advise on how serious is blood vomit, what are the today's technological options and medical treatment.
Posted Mon, 21 May 2012 in Congenital Heart Disease
Answered by Dr. Raja Sekhar Varma 3 hours later
Thank you for your query.

As I understand, the patient is a 38 year old lady with a Ventricular Septal Defect which is shunting bidirectionally due to severe pulmonary arterial hypertension. This is also known as Eisenmenger's syndrome.

As it stands, once this stage of the disease is reached, simple, surgical closure of the hole (VSD) is impossible.

The only "permanent" form of treatment is a "heart-lung" transplantation or single lung transplantation with surgical repair of the VSD, which obviously is very expensive, not widely available and has other multiple and obvious limitations.

Palliative treatment is the only realistic option for most patients. This includes a salt-restricted diet, regular mild exercise in the form of walking (no strenuous activity is allowed), rehabilitative cardiac physiotherapy, and drugs. Drugs like digoxin, low dose diuretics are useful to relieve symptoms. There are some novel drugs which can help to reduce the pulmonary vascular resistance and improve the flow of blood to the lungs. These include drugs like sildenafil and tadalafil. More specific but very expensive drugs include bosentan and ambrisentan (both are available in India). The doses have to be individualized and titrated under close supervision to achieve the best possible results.

It is also important to monitor the Hemoglobin and Packed Cell Volume values. If the values are high, periodic blood-letting (phlebotomy) and removal of a calculated volume of blood with replacement by other fluids is important to prevent the hyper-viscous blood from forming clots and blocking small blood vessels and capillaries.

It is also important to prevent dehydration at all times.

Bleeding is also an important and frequent complication that has to be addressed carefully. Immediate attention and appropriate treatment including local measures and transfusions of plasma, cryoprecipitate and platelets may be urgently needed.

One has to also closely watch out for complications like Infective Endocarditis and complications of blood clots bypassing the lungs and going to the brain, etc causing 'paradoxical' embolism. Pregnancy is completely contra-indicated.

I hope this answers your query. Feel free to ask for any specific clarifications.
With regards,
Dr RS Varma
Above answer was peer-reviewed by
Follow-up: Diagnosed with Congenital heart disease, situs solitus, mid muscular VSD bidirectional shunt. Had blood vomit. Serious? 7 hours later
You have mentioned about the HB AND Packed cell volume. It measures at 16.3 gm/dl and packed cell volume is 49.8. Platelet count is 255. Please advise the suggested course of action.
Answered by Dr. Raja Sekhar Varma 20 hours later
Thank you for the reply.

With a hemoglobin value of 16.3 and PCV of 49.8, phlebotomy is not needed at present unless there are symptoms like persistent headache, giddiness or breathing difficulty.

Once the Hb reaches 18 or more, and PCV approaches 55, you can plan for phlebotomy. Initially, monthly monitoring may be needed. Once the rate of increase of Hb is identified, the frequency of check-up can be decided.

The blood volume removed is usually replaced with Fresh Frozen Plasma or saline.

The platelet count is normal.

I hope this answers your query.
With regards,
Dr RS Varma
Above answer was peer-reviewed by
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