Young women with mitral valve disease, atrial fibrillation or not and heart failure. What will be discussed in the viva voce examination.

Scenario 1.

A 16 year old girl is brought by her family to a tertiary care cardiac facility for management. She has been unwell for 5 years with severe palpitation, breathlessness which is exertional, recurrent swelling of the face and feet and has needed hospitalisation for worsening of her symptoms, to the local hospitals near the village where she lives. Her family have been told that that she has a cardiac valve disease in which the valve is tight and leaky and that she needs surgery for it’s correction. She comes from a poor family and until now they have decided against her surgery. Her brothers are now working so the family decided to come to the city for her surgery as their financial circumstances now permit them to afford a stay in the city. You assess her for further management.

These are the problems that you see:

  • She is in heart failure. She is severely breathless, tachypnea and orthopneic; prefers to sit up. Her feet and face are swollen, she has some ascites, a raised JVP, bibasilar creps, an enlarged liver which is pulsatile. Her praecordium is bulging and very pulsatile. The apex beat is in the anterior axillary line. This indicates that the cardiomegaly has been there for some years pushing out the sternum.
  • The pulmonary hypertension may be irreversible making the prognosis poor.
  • The pulse is rapid 104/min at rest and irregular. BP is 100/65 mm Hg. She is fibrillating and has lost the 25% boost that a contracting atrium give to the diastolic pulse. She may throw an embolus.
  • Cardiac auscultation shows variable intensity of the first heart sound, a pansystolic murmur all over the precordium, with a loud second pulmonary sound and an irregular heart beat. She has a mitral valve which cannot close properly so is incompetent, causing blood to regurgitate back into the left atrium when the left ventricle contracts. She has pulmonary hypertension indicating that the mitral valve was/is stenosed as well and she is fibrillating. As her liver is enlarged and pulsatile and the regurgitant murmur is prominent over the tricuspid, there is tricuspid regurgitation which is probably functional.

What problems will you look for if you are evaluating her for possible valve replacement surgery?

  • Has she got active carditis? You are going to be asked how you will evaluate her for carditis so be prepared.
  • Has she got a clot in her left atrium and does she need anticoagulation? Is anticoagulation likely to reduce her risk of embolisation even if she does not have a clot? These points will be discussed so know the answer.
  • Can her AF be converted to sinus rhythm or can she be managed by rate control?
  • Does she have an enlarged left atrial appendage? Is removal of an enlarged left atrial appendage likely to reduce the incidence of AF post operatively?
  • What therapy does she need to improve her cardiac status and ejection fraction and tricuspid regurgitation? Remember that ACE-I are not useful in valvular heart disease.
  • Is her pulmonary hypertension reversible or fixed?
  • Does she have bacterial endocarditis and vegetations? How will you assess her for endocarditis?
  • What is the status of her aortic valves?
  • Which prosthetic valves are suitable for valvular replacement in this patient?
  • Will she be able to have a baby once she has her new valve? How do you manage a pregnancy in a patient with a valvular heart disease?

When you assess her you have to keep these points in mind. You will definitely need to keep these points in mind when planning her management as well as for counselling her about her prognosis.

Scenario 2.

A 27-year-old primigravid woman at 25 weeks 5 days of gestation presented to the emergency department in respiratory distress. She had been well until the evening before, when a cough productive of blood-tinged, frothy sputum had developed and worsened progressively. She noted shortness of breath and chest pain but no fevers, chills, hemoptysis, lower-extremity edema, or calf pain. She reported regular fetal movement and no uterine contractions.

  • What is the significance of calf pain? Pregnancy is a hypercoagulable state so think of deep vein thrombosis in the legs.
  • Why is she breathless? The cause may be similar to a non-pregnant woman which may include community-acquired pneumonia, asthma exacerbation, pulmonary embolism, and cardiogenic or noncardiogenic pulmonary edema.
  • Look for cardiovascular causes of pulmonary edema in pregnancy which include preeclampsia with severe asymptomatic hypertension or hypertensive emergency, valvular heart disease, and cardiomyopathy.
  • Peripartum cardiomyopathy would not be expected at this stage of pregnancy, nor would amniotic-fluid embolism.
  • Why is it necessary to ask about uterine contractions? Chorioamnionitis-induced acute respiratory distress syndrome is rare in the absence of premature labor or ruptured membranes.

This patient comes from the Indian subcontinent. The incidence of rheumatic heart disease is still common. She may give a history of sore throat, or fever associated with swollen painful joints but 25% have no history and at least 25% have forgotten. Ask if she had to take monthly injections as prophylaxis which she is more likely to remember. Also ask if she has had episodes of palpitation or breathlessness in the past.

She was in acute respiratory distress and sitting up; she was coughing and using accessory muscles of ventilation. The temperature was 37.4°C, the pulse 144 beats per minute and regular, the blood pressure 142/80 mm Hg, and the respiratory rate 30 breaths per minute. The oxygen saturation was 76% while the patient was breathing ambient air and increased to 85% with the use of a nonrebreather face mask delivering 70% oxygen. The jugular venous pressure was elevated to the angle of the jaw. Chest examination revealed diffuse rales bilaterally. Cardiac examination revealed tachycardia with a left parasternal lift and a prominent pulmonic component of the second heart sound (P2); no murmurs were detected. The abdomen was normal. The fundal height was 23 cm. The fetal heart rate was 130 to 140 beats per minute. The cervix was closed. The extremities were warm, pedal pulses were 2+ bilaterally, and there was no edema.

The ECG showed sinus rhythm and P mitrale. This indicates an enlarged left atrium.

An ultrasound of the abdomen was deferred because of he respiratory distress.

Urgent bedside echocardiography showed changes consistent with rheumatic mitral-valve disease with predominant stenosis, chordal thickening, restricted leaflet motion, and a “hockey stick” appearance of the anterior leaflet. The mean mitral-valve pressure gradient was 20 mm Hg at a heart rate of 140 beats per minute. Mitral regurgitation was qualitatively mild. The left atrium was severely enlarged (volume index, 80.74 ml per square meter). The systolic pressure in the pulmonary artery was markedly elevated (95 mm Hg). Right ventricular hypertrophy and signs of pressure overload were present. The size and thickness of the left ventricle were normal, as was systolic function.

The findings are consistent with severe rheumatic mitral stenosis, complicated by advanced pulmonary hypertension and right ventricular pressure overload.

This is her Xray chest.

Her primary care physician after blood, urine, and sputum cultures had been obtained, administered intravenous furosemide, methylprednisolone, vancomycin, and ceftazidime and transferred her to a tertiary care institution for further management of her condition. Why was she given steroids? What is the use of antibiotics? Why steroids?

The findings are consistent with severe rheumatic mitral stenosis, complicated by advanced pulmonary hypertension and right ventricular pressure overload. In pregnancy, even low degrees of mitral stenosis can be associated with increased maternal and fetal morbidity and mortality, because the dilutional anemia and decreased plasma oncotic pressure of pregnancy may promote pulmonary edema at lower hydrostatic pressures.

What are the triggers which have set off the pulmonary edema and increased the pulmonary hypertension?

  • Hypervolemia of pregnancy.
  • Increased circulation from the pregnant uterus.
  • Reduction in vital capacity from an enlarging uterus.
  • Dilutional anemia of pregnancy.
  • Decreased plasma oncotic pressure in pregnancy

In the majority of pregnant women with symptomatic severe mitral stenosis, heart-rate control and judicious diuretic administration restore hemodynamic stability. When these measures failed, valve intervention was considered. Percutaneous balloon mitral valvuloplasty is the preferred intervention and was performed in this patient.

What kind of delivery be planned in severe or even moderate mitral stenosis?

Vaginal delivery with epidural anesthesia is usually recommended; a cesarean section is performed for obstetrical indications and in patients with the most precarious hemodynamic status. The Valsalva maneuver may be poorly tolerated, because it transiently decreases preload and causes compensatory tachycardia. The second stage of labor should therefore be shortened when possible. The stress of the Valsalva maneuver may be alleviated by allowing the fetal head to passively move downward during the second stage of labor, reducing the amount of time spent pushing. If there is hemodynamic instability, delivery may be assisted with the use of forceps or vacuum extraction. Most patients do not require invasive hemodynamic monitoring during delivery.

So pregnancy is not only possible with valve disease, the valve lesion is usually detected after the pregnancy has been established as the hemodynamic changes in pregnancy set off heart failure.

Scenario 3.

A 32 year old woman was admitted in a tertiary care hospital with gross enlargement of her abdomen, had pain on the right side of the abdomen for the last 10 days. She was severely breathless so that she had difficulty in talking so was able to give only a brief history, coughed up frothy sputum, could not lie flat on her bed, had swollen feet. When her distress was relieved by giving IV diuretic, oxygen and opioid injection she gave the history that she had been unable to do any housework for the past 3 months with her 12 year old daughter doing the cooking and cleaning. with a neighbour coming in to look after her toilet needs. Sha had had 3 pregnancies and her youngest child was 11 months old. At each pregnancy she became severely breathless and bedridden. She had not been able to come to a hospital for an antenatal examination and her pregnancy and deliveries had been managed by the village midwife. All three had been vaginal deliveries.

She had gross ascites, was not jaundiced, her liver was enlarged by 9 centimeters below the costal margin and her spleen was 6 cm and firm in consistency. She had no markers of chronic liver disease. Her pulse was 120/min, regular, BP was 90/60 mmHg, respiratory rate was 30/min, she was afebrile. Her JVP was markedly raised, there was edema up to mid thighs, her precordium was hyperdynamic with a palpable systolic thrill all over the precordium, the cardiac apex was in the midclavicular line on the left side. The first heart sound was soft, with a pansystolic murmur almost drowning the both heart sound but in the pulmonary area P2 could be heard quite distinctly. There was no early diastolic or mid diastolic murmur or an opening snap. There were marked crepitations all over the chest with evidence of bilateral pleural effusion.

What is unusual about her presentation and findings? She is obviously in advanced heart failure hut why is the fluid retention localising to her peritoneal cavity, why is the liver painful and tender and what is the significance of the enlarged spleen?

What is the difference between a firm spleen and a soft spleen? A soft spleen has recently been enlarged so can occur in fevers such as endocarditis, typhoid fever, recent onset malaria, brucellosis etc. A firm spleen has been there for weeks or months so think of chronic conditions like portal hypertension, disseminated TB, chronic leukemia, lymphoma etc.

The liver is painful because of recent and sudden onset of enlargement of the liver. Possible rapid decompensation of the valvular heart disease has caused TR and the liver size has gone up suddenly. The spleen indicates that the portal pressure is rising and is being transmitted via the portal vein the portal system. The patient may have early onset of cardiac cirrhosis not just congestive cardiomegaly. She has no fever but has endocarditis damaged the valve further caused more pressure on th right or left ventricle causing further decompensation?

As you can see that each patient has something subtly different because the disease is progressing differently. You have to sort out which problem is more important in every patient. You have to remember that you are not treating a valve lesion but a valve lesion which is causing different difficulties in different people so each patient needs an assessment that takes into all the different factors.

I have given you plenty of points for thought. The candidates who are not able to discuss these points are not going to survive in a post graduate assessment.

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I am a Professor of Medicine and a Nephrologist. Having served in the Army Medical College, Pakistan Army for 27 years I eventually became the Dean and Principal of the Bahria University Medical and Dental College Karachi from where I retired in 2016. My passion is teaching and mentoring young doctors. I am associated with the College of Physicians and Surgeons Pakistan as a Fellow and an examiner. I find that many young doctors make mistakes because they do not understand how they should answer questions; basically they do not understand why a question is being asked. My aim is to help them process the information they acquire as part of their education to answer questions, pass examinations and to best take care of patients without supervision of a consultant. Read my blog, interact and ask questions so that I can help you more.

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