A 51-year old woman was brought to the emergency of a tertiary care hospital with severe breathlessness, cough made worse by lying down, with sputum production tinged with blood. She has had these symptoms for 8 months.
The patient has had 3 episodes of palpitation and fainting and has progressively become breathless in the past 3 years for which she was treated in a dispensary in her village manned by a male nurse. She also has episodic chest pain lasting for 5 to 10 minutes brought on by exertion and relieved by rest also for 3 years. When resting she prefers to sit on the edge of a charpoy or bed leaning forward. To facilitate her, her husband has bought her a cane chair as they have no chairs in their home. She has had bouts of cough with some blood in her sputum for 7 or 8 months. She had received little or no treatment until recently and is now admitted in a semi-private room as a paying patient. Her husband is a laborer in the fields in the village and has an income which barely provides food for the family to eat. She has had 4 pregnancies all resulting in live births. Her oldest daughter is 22-years old and married, two sons are also laborers in the village and the youngest daughter was born 10 years ago. She did not have symptoms of syncope or breathlessness during any pregnancy and received no ante-natal care except for that provided by a dai (a self trained village mid-wife). Her childhood has been uneventful, not marked by a significant febrile event that she remembers. She has had frequent attacks of upper respiratory infections in recent years and has generally been tired and unwell since her last pregnancy with her oldest daughter doing most of the house work. Her diet is mostly vegetables and chapatti (flour flatbread) and boiled rice. She does have access to dairy such as milk, cream, yogurt and lassi (yogurt drink like Turkish airan) and desi ghee (clarified butter) as her husband bought a cow a few years ago.
The portions in italics were missed by the candidate when taking the history. They are part of the socio-economic history.
On clinical examination her pulse was 90/min, irregular without a pattern to the rhythm, BP was 110/65 mm Hg, there was pedal edema, a raised JVP up to the angle of the jaw, she was not cyanosed or anemic or jaundiced. There was a mild wheeze in her chest with vesicular breath sounds. There were crepitations in both lung bases. There was no cardiomegaly with a rather indistinct and tapping apex beat. There was no right ventricular heave. The first heart sound was loud and distinct and best heart at the apex with an opening snap, the second heart sound was louder at the pulmonary area but had a good radiation to all the cardiac auscultatory areas. There was a mid diastolic murmur detectable when asking the patient to turn toward the left arm and listening with care, which did not accentuate towards the end or radiate anywhere. A faint short mid-systolic murmur could be heard. There were no third or fourth heart sounds. The liver was enlarged by three fingers, soft and not tender. The spleen was not palpable and there was no peritoneal fluid detectable. There was no neurological deficit. She has gained weight since her last pregnancy.
- What is the clinical diagnosis? What is the differential diagnosis?
- She appears to have mitral valve stenosis, probably of rheumatic origin, along with significant pulmonary hypertension causing pulmonary edema, and atrial fibrillation. She has had not had an embolic event.
- The differential diagnosis includes left atrial myxoma and cor triatriatum. Cor triatriatum (division of the left or right atrium by a membrane that may cause obstruction to flow) is diagnosed by echocardiography. Left atrial myxoma is unlikely as she does not have a significant history of fever and weight loss. Both the conditions can be differentiated by doing an echocardiogram.
- How will you determine that the valve lesion is of rheumatic origin?
- There are no definite tests to prove that this valve lesion is of rheumatic origin.
- Mitral stenosis of >4mm gradient without calcification, MS and pathological MR (not functional) are likely to be of rheumatic origin.
- How did she have 4 uneventful pregnancies? the following could be the reason:
- She may have developed rheumatic carditis after her last pregnancy as it is possible to get rheumatic fever later in life but it is commoner in people before the age of 40. She has had URI frequently in the last ten years with possible exposure to streptococci.
- If she already had mitral stenosis before her first pregnancy it was not a tight stenosis and so she tolerated the increased blood flow and increased blood volume in pregnancy. In stage A there is tendency to doming of the mitral valve but the blood flow across the valve is less than 150 ms. This progresses to MS with commissural fusion and significant doming but the valve area is more than 1.5 cm squared. It is when the area of the valve is less than 1.5 cm squared and trans-chamber blood flow is around 220 ms that patients develop clinical signs of mitral stenosis i.e stage D. Progression to stage D may take up to 10 years.
- She had no other valve involved so mitral stenosis was tolerated better.
- Her left atrium was not significantly enlarged so she did not have AF in the pregnancies. AF causes loss of 25% of blood flow from the left atrium to the left ventricle and to poor cardiac output.
- The pulmonary hypertension had not developed to a degree to cause a pulmonary capillary wedge pressure of 10 mm of water so pulmonary edema did not develop during pregnancy.
- How is she admitted in an expensive hospital when her husband could not afford even basic treatment?
- This point has been significantly missed in the clinical clerking or patient interview. Her husband had probably not dug up treasure in his field but may have sold his land for her treatment but then he would have sought treatment in a government facility which is much cheaper. What is more likely is that a relative, who is well off economically, has intervened. A brother or sister returning from a job in UAE or USA is likely to have taken over medical responsibility. You need to find out.
- Explain the episodes of syncope?
- It could be the onset of an arrhythmia: atrial fibrillation or less likely SVT.
- The tight mitral stenosis itself can cause syncope as well as pulmonary hypertension.
- Advanced heart failure can cause syncope.
- Vasovagal syncope. It would not be wise to accept it only as a reflex syncope as she is a high risk patient, A careful assessment of her cardiac status
- Why does she have angina like pain?
- Patients with severe pulmonary hypertension have pain resembling angina pectoris because of right ventricular hypertrophy which develops as the right ventricle has to contract against increasing pulmonary pressure.
- Does she need an exercise tolerance test? She needs a stress echocardiogram not a stress related ECG. Stress (exercise or dobutamine) echocardiography is particularly helpful when symptoms seem to be more severe than expected based on resting hemodynamics (such as resting valve area); these patients often have exertional pulmonary hypertension that can be detected on stress echocardiogram.
- The widespread availability of echocardiography has led to a limited role for cardiac catheterization in the diagnosis of MS. However, invasive pressure measurements are warranted if noninvasive tests are not conclusive, or there is a discrepancy between noninvasive tests and clinical findings or between clinical symptoms and hemodynamics at rest, or severity of pulmonary arterial hypertension is out of proportion to the severity of MS as determined by noninvasive tests
- Explain her auscultatory findings in the heart.
- The loud first heart sound is caused by the stenotic (but non calcified) mitral leaflets which are still widely separated at the onset of ventricular contraction. Thus, the first heart sound (S1) is loud, reflecting the increased excursion of the stiff leaflets. As leaflets become less mobile and more calcified the first heart sound becomes softer. Hence a loud first heart sound indicates mobile or non-calcified mitral valve leaflets. The opening snap (which is not present in this patient) also indicates mobile non-calcified mitral valve leaflets. It is best heard at the apex of the heart. the distance in time between the OS and the second heart sound is a guide to the degree of stenosis. The closer together they are the more severe the stenosis is likely to be. The second heart sound becomes loud and is always single in significant pulmonary hypertension. It is best heard in the pulmonary area in inspiration but a loud P2 can be heard all over the praecordium. The mid systolic murmur is the Graham Steell murmur caused by increased flow of blood across the pulmonary valve. The mid-diastolic murmur is the characteristic murmur of MS, best heard in a quiet room with the patient turning over to the left side in expiration. In the absence of AF there is pre-systolic accentuation.
- Why is the mid-diastolic murmur not accentuating towards the beginning of systole?
- This is because there AF hence the atrial flow into the ventricle is not augmented at the end of ventricular diastole.
- How will you treat the arrhythmia and prevent the recurrent syncope?
- How will you stage mitral stenosis?
STAGING (taken from Uptodate)
Based on integration of information about valve anatomy, valve hemodynamics, secondary hemodynamic effects, and patient symptoms, MS severity is graded as follows :
●Stage A includes patients at risk for MS. Features include mild mitral valve diastolic doming and normal transmitral flow velocities.
●Stage B includes asymptomatic patients with progressive MS. Rheumatic mitral valve changes include commissural fusion and diastolic doming. Transmitral flow velocities are increased but the mitral valve area is >1.5 cm2 and diastolic pressure half-time is <150 ms. Mild to moderate left atrial enlargement is present and pulmonary artery pressures are normal at rest.
●Stage C includes asymptomatic patients with severe MS defined as a mitral valve area ≤1.5 cm2 and diastolic pressure half-time ≥150 ms. Rheumatic mitral valve changes are present along with severe left atrial enlargement and pulmonary artery systolic pressure >30 mm Hg.
This category includes asymptomatic patients with very severe MS, defined by a mitral valve area ≤1.0 cm2 and diastolic pressure half-time ≥220 ms.
●Stage D, includes symptomatic severe MS defined as a mitral valve area ≤1.5 cm2 and diastolic pressure half-time ≥150 ms. Rheumatic mitral valve changes are present along with severe left atrial enlargement and pulmonary artery systolic pressure >30 mmHg. This category includes symptomatic patients with very severe MS, defined by a mitral valve area ≤1.0 cm2 and diastolic pressure half-time ≥220 ms.
This is a pulmonary angiogram of a patient with severe hypertension. It show upper lobe blood diversion or cephalisation of pulmonary vasculature with the lower fields almost complete lack of visualisation of the blood vessels usually labelled pruning of the pulmonary vasculature as if the branches of a tree have been lopped off.. The heart is enlarged with a prominent left atrium which may be seen to impinge on the esophagus on a lateral view.