MJ Is a 24 Year Old Female (HIV -) with a History of Congestive Heart Failure (Unknown

MJ is a 24 year old female (HIV -) with a history of congestive heart failure (unknown etiology) who presented to Kamuzu Central Hospital (KCH) with complaints of abdominal distension and discomfort for 3 years and 1 month of dyspnea/cough/heart palpitations. She denied any orthopnea, PND, fevers, night sweats, or weight loss. She had previously had many episodes of similar complaints, each time requiring paracentesis, thoracentesis, and pericardiocentesis (including 6 previous admissions at KCH).

On arrival, her physical examination was remarkable for temperature of 35.6C (afebrile), pulse/BP that were not palpable/recordable, distended neck veins, muffled heart sounds, bilateral basilar crackles on lung exam, globally distended/fluid filled abdomen, and bilateral pitting edema in her lower extremities. An abdominal ultrasound showed ascites, tricuspid stenosis, right atrial dilation, dilated IVC/hepatic veins, and a massive pericardial effusion leading to tamponade/poor ventricular function. Pericardiocentesis was performed, draining 6L of green fluid. Bilateral thoracenteses were also performed. She was started on Furosemide 40mg OD and ASA 75mg OD. Enalapril was considered, but held as BPs (when recordable) were low (80/50). Ascitic tap the day after admission drained 2L of yellow fluid.

Several diagnostic studies were performed during the course of her hospital stay. Chest X-Ray showed bilateral pleural effusions and a massive cardiac shadow. Pericardial and pleural fluid were sent for diagnosis and returned unremarkable (including being unconcerning for TB/other infection). TTE showed normal LV (EF 55-60%)/LA/AV/MV, “smoke” indicative of a thrombus in RA/RV, severely dilated RA, severe tricuspid stenosis, and a large pericardial effusion (4-6cm). The triscuspid stenosis was felt to be congenital, as there was no sign of mitral involvement (and, therefore, highly unlikely to be secondary to rheumatic heart disease).

Following the taps (pericardial, pleural, and abdominal), the patient improved symptomatically as LV function returned to normal (as evidenced by TTE results). Her blood pressure normalized (100s-110s/60s-70s). She had one more pericardiocentesis (1L of straw colored fluid) and was discharged on her 7th day of admission on 75mg OD furosemide (for symptomatic relief from her heart failure) and 40mg OD aspirin (palliative for the thrombus seen on TTE).

A few questions were left unanswered for the patient after discharge. First of all, it is confusing why she was discharged without being placed on an ACE-I, which is standard treatment for any kind of CHF. In addition, the patient had isolated right sided heart failure, which explains her recurrent ascites and peripheral edema. However, there was not a satisfactory explanation given for her pleural effusions/dyspnea/cough (signs and symptoms of left sided failure) or her recurrent pericardial effusions. It is possible that the pericardial effusions were intermittently getting large enough to cause tamponande and the rest of her symptoms, but the underlying cause of the pericardial effusions was still unexplained. Several possibilities were considered for the cause of her pericardial effusions. Autoimmune diseases such as SLE are one potential cause, but she denied all other symptoms, such as malar rash, photosensitivity, neurological symptoms, hematological problems, joint pain/inflammation, etc. An empiric trial of steroids could have been considered, but there are no real rheumatological diagnostic tests available at KCH. TB is always high on the differential due to its prevalence, but diagnostic taps were negative. However, these are notoriously insensitive for TB, and the patient could have been started on empiric TB treatment and followed up for response. Finally, it is possible that what was thought to be a thrombus on TTE may in fact be a myxoma or other tumor, which would explain both the tricuspid stenosis and pericardial effusion. Unfortunately, if it was a myxoma, the definitive treatment would be surgery, which is not an option (as explained later).

There are several options for treatment of isolated tricuspid stenosis and subsequent right sided CHF. These include medical management (ACE-I/diuretics), balloon valvotomy, and surgery. At this point, valvotomy is the gold standard for treatment, as it has the best long-term outcomes (valve area, NYHA Class, restenosis rates, reintervention rates). Therefore, most patients in MJ’s situation (severe valvular stenosis with CHF) in the US would undergo valvotomy.

However, valvotomies are not performed in Malawi, as the resources are not available. Neither are surgeries. In order to qualify for these procedures, the patients first have to prove that they have a good social situation and appropriate support and that they are likely to have good medical follow up both prior to and following the procedures. They are then placed on a waiting list to be flown out of the country (usually either to India or South Africa) to undergo the procedures, with the Malawian Ministry of Health providing financial support. However, the list is long and government funds are limited. Unfortunately, MJ was judged to be unlikely to have good medical follow up and did not even make it onto the wait list.

This frustration with lack of resources was a common theme at KCH. While most of the time the frustration came with lack of diagnostic capabilities (labs, CT scans, etc), in CHF patients, the problem was often with lack of capability for intervention. This is particularly true now that there is an American cardiology fellow who performs Echo’s regularly. He is able to see many patients, diagnose CHF, and, often, the underlying etiology of the CHF. However, with almost all of the diagnoses, there is no procedural intervention possible, and the patients are discharged with the same medical management for CHF they would have received, even without a specific diagnosis. There is great need and opportunity for establishing better access to cardiac interventions for the people of Malawi.