Online Clinical Case Study (July 2005)

Clinical Cardiology Series
臨床心臟科個案研究

The content of the Office Cardiology Series is provided by:
Dr. Yiu Siu Fung
M.B.,B.S.(H.K.), M.R.C.P.(U.K.), F.H.K.A.M., F.H.K.C.P., F.R.C.P.(Glasg.), F.R.C.P. (Edin.)
Dr. Li Siu Lung, Steven
M.B.,B.S.(H.K.), M.R.C.P.(U.K.), F.H.K.A.M., F.H.K.C.P., F.R.C.P.(Glasg.), F.R.C.P.(Edin.), Specialist in Cardiology
Dr. Wong Shou Pang, Alexander
F.R.C.P., F.H.K.A.M.(Med.), F.H.K.C.P., Specialist in Cardiology
臨床心臟科個案研究之內容誠蒙姚少峰醫生、李少隆醫生王壽鵬醫生提供。

A 32 years old lady attended your clinic for unexplained cardiomegaly on routine chest X-ray. She was asymptomatic and remained in Functional Class I all along. Physical examination revealed the presence of an apical grade 3/6 pansystolic murmur radiating to the axilla. ECG showed normal sinus rhythm with left ventricular hypertrophy by voltage criteria. Her chest Xray showed cardiomegaly, but the lung fields were clear. She was referred for transthoracic echocardiography examination, which confirmed your examination finding of severe mitral regurgitation (MR) due to prolapse of the anterior mitral valve leaflet. The left ventricle (LV) was dilated with end systolic dimension measuring 42mm. However the LV systolic function was normal with an ejection fraction (EF) measuring 65%. There was no evidence of pulmonary hypertension. She was worried and enquired on management plan and whether she could become pregnant.

 

Answers

1.

Which of the following management should be recommended?

1. No definite contraindication for pregnancy, start angiotensin converting enzyme inhibitor (ACEI), withhold treatment once she becomes pregnant with more frequent echocardiographic monitoring during pregnancy

2. Same as above except withholding ACEI therapy before pregnancy

3. Advise against pregnancy, and refer her for early mitral valve surgery

4. Advise against pregnancy, medical therapy with ACEI, defer surgery unless serial echocardiographic studies show further LV dilatation

The Answer is 3

Unlike other valvular lesions,chronic asymptomatic severe MR is a deceptive lesion for both the patient and the physician. Management is far more difficult than any other valvular lesion and the following facts should be remembered when patients with chronic severe organic MR are encountered.

1.Surgery is always the definitive treatment modality for severe MR.

2.There is no controlled trials comparing the effect of medical treatment versus surgical therapy in patients who have severe chronic asymptomatic MR.Designing such trials are difficult since MR is a heterogeneous disease in terms of severity and etiology.Medications (ACEI or beta blocker) do not pose survival benefit in these patients.Though ACEI can be prescribed in patients with left ventricular dysfunction,the teratogenic side effects must be explained and documented to all female patients of child bearing age for medico-legal purpose.

3.Organic severe MR is not a benign disease.Excessive mortality and morbidity (atrial fibrillation,heart failure)is noted and surgery is nearly unavoidable during long term follow up.No group at very low risk under medical treatment can be defined (NEJM 1996; 335:1417-23)

4.Sudden death is a catastrophic event,responsible for about a quarter of the deaths occurring under medical treatment (JACC 1999; 34: 2078-85)

5.MR is a progressive disease.However the rate of progression is not uniform and is unpredictable (JACC 1999; 34:1137-44)

6.Infective endocarditis is a serious complication which can cause rapid downhill course.Remember to educate your patient the importance of antibiotic cover during invasive procedures.

7.MR causes increase in preload &decrease in afterload, therefore LV function often looks better than true myocardial function.Postoperatively EF immediately decreases by approximately 10%with increase in afterload.

8.Survival after mitral valve repair is always better than mitral valve replacement if feasible (Circulation 1995; 91: 1022-1028)

9.Significant calcification,rheumatic etiology,or endocarditis are factors unfavorable for mitral valve repair.Posterior mitral valve leaflet prolapse is always easier to be repaired than anterior or bileaflet mitral valve prolapse.

10.CXR monitoring of cardiothoracic ratio is not reliable to guide the presence or absence of LV dysfunction.

11.Two main echocardiographic parameters to consider mitral valve surgery in the absence of cardiac symptoms are:(1) evidence of LV dysfunction with LV end systolic dimension >= 40mm (normal <36mm)and / or LVEF <= 60%(normal > 60%)(2)pulmonary hypertension with resting pulmonary artery systolic pressure >50mmHg (normal around 30-35mmHg).

12.Due to smaller body built of Chinese population comparing with Caucasians,we usually advocate 40mm and not 45mm as the cutoff line for surgical referral.

13.If the valve can be repaired with low operative risk (<=1%), development of paroxysmal AF,massive degree of MR (regurgitant volume >100ml),severe left atrial dilatation (M mode dimension >45mm),abnormal EF response to exercise can also be indications to consider valvular surgery.

14.Symptoms of the patient always override echocardiographic parameters in decision for surgery.If a patient has functional class deterioration or unexplained heart failure,surgery should be advised even in the absence of LV dysfunction or pulmonary hypertension by echocardiography.Similarly if a patient has impaired LV function during heart failure hospitalization but recovers fully during follow up echo,he or she should be referred for early surgery.The ※normal § LV size and function is just a delusion and these patients frequently suffer from post-operative LV dysfunction.

15.Although regurgitant lesions are better tolerated than stenotic valvular lesions during pregnancy,presence of preexisting LV dysfunction poses extremely high risk for maternal heart failure and death during pregnancy. Therefore it should be avoided.The valve should be repaired or replaced with bioprosthesis before pregnancy. If the patient intends for pregnancy after cardiac surgery, mechanical valve replacement should be avoided due to the need for warfarin postoperatively.

In summary,chronic severe organic MR is not a benign disease. If the golden time for surgery has passed,the patient will suffer from irreversible LV dysfunction despite corrective mitral valve surgery.Early LV dysfunction and pulmonary hypertension are the two main indications for early surgery in the absence of cardiac symptoms which can be determined accurately by echocardiography.The role of ACEI or beta blocker in severe MR remains undetermined.

 

Back to top

 


Dermatology Series 皮膚科病例研究

A 45 year old Chinese male presented with asymptomatic skin rash over his legs for two months. There were no systemic signs and symptoms. His general health was good and there was no long-term drug. Physical examination revealed multiple non-palpable tiny red dots localized on his legs. Laboratory investigations were normal.

The content of the Dermatology Series is provided by:
Dr. Chan Loi Yuen and Dr. Tang Yuk Ming, William
Specialist in Dermatology & Venereology

皮膚科病例研究之內容誠蒙陳來源醫生鄧旭明醫生提供。

Answers

1.

What is the clinical diagnosis?

The diagnosis is Schamberg's disease (most common type of pigmented purpuric dermatoses).It is a form of capillaritis.There is erythrocyte breaking down outside the capillary and leaving hemosiderin deposits.The typical lesions are pinhead-size,reddish puncta resembling "Cayenne pepper spots". After a few months the lesions begin to fade into the surrounding pigmented patches. The lesions are seldom itchy.

 

Back to top

 

2.

What are the clinical differential diagnoses?

Clinical differential diagnoses for multiple petechial rash include Henoch-Schenlein purpura,idiopathic thrombocytopenic purpura and cutaneous T-cell lymphoma.

 

Back to top

 

3.

What is the pathogenesis?

The cause is unknown.It may be due to cell-mediated immune response or increased capillary fragility.Venous hypertension,exercise and gravitational dependency are cofactors.It may also be due to drugs such as aspirin and thiamine.

 

Back to top

 

4.

What are the investigations?

Laboratory investigations include complete blood count, erythrocyte sedimentation rate and clotting studies.Skin biopsy may be required and show capillaritis in the upper dermal vessels,extravasation of the red blood cells and haemosiderin deposits in the macrophages.

 

Back to top

 

5.

What are the treatments?

Treatment may not be required if there is no symptom. Prolonged leg dependence should be avoided.Topical steroid may be used if there is mild itch.Graduated compression elastic hose may be helpful. Photochemotherapy and systemic steroid are effective in clearing the lesions but the risk usually outweighs the benefit.Pentoxifylline and ascorbic acid has been reported to be useful.

 

Back to top

 

Back to Online Clinical Case Study