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 Table of Contents  
MISCELLANEOUS - BEDSIDE CASE
Year : 2015  |  Volume : 1  |  Issue : 3  |  Page : 276-280

A case of valvular heart disease


Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication23-Feb-2016

Correspondence Address:
Vijay Bohra
All India Institute of Medical Sciences, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2395-5414.177291

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  Abstract 

A 29-year-old female had presented with dyspnea on exertion since she was 10 years old, with sudden worsening for 5 months along with palpitations on exertion for the past 5 months. The examination findings, electrocardiogram, chest X-ray, and echocardiogram are discussed in a stepwise manner to arrive at a diagnosis and plan the management of a patient with rheumatic heart disease with multivalvular lesions. Relevant literature is also reviewed.

Keywords: Bedside case discussion, clinical case, rheumatic heart disease


How to cite this article:
Bohra V, Ramakrishnan S, Parakh N. A case of valvular heart disease. J Pract Cardiovasc Sci 2015;1:276-80

How to cite this URL:
Bohra V, Ramakrishnan S, Parakh N. A case of valvular heart disease. J Pract Cardiovasc Sci [serial online] 2015 [cited 2019 Jul 20];1:276-80. Available from: http://www.j-pcs.org/text.asp?2015/1/3/276/177291


  Clinical Presentation Top


A 29-year-old lady presented with:

  • Dyspnea on exertion since she was 10 years old, sudden worsening for 5 months
  • Palpitations on exertion for past 5 months.


The patient was apparently well until 10 years of age when she started experiencing dyspnea on exertion, which was insidious in onset and gradually progressive. It was relieved with rest, and there was no paroxysmal nocturnal dyspnea (PND) or orthopnea. There was worsening in dyspnea in the past 5 months with PND and orthopnea. At present, she can walk only 250-300 meters at a stretch.

She also gave a history of palpitations for the past 5 months. These were present at rest and aggravated during exertion. There was no history of similar palpitations in the past or syncope. There was no history of a chronic cough, hemoptysis, fever or any history of pedal edema, and right upper quadrant pain abdomen.

There is no history of polyarthralgia or arthritis, rash, involuntary movements, painful digits, or hospitalization. There is no history of addiction or drug abuse. She receives an intramuscular injection every 3 weekly since the onset of illness along with few other oral drugs which she is unable to identify. She denies history of similar illness in the family.


  Summary Top


A 29-year-old lady presented with insidious onset gradually progressive dyspnea on exertion since she was 10 years old with rapid recent worsening for the past 5 months associated with palpitations at rest.


  Discussion Top


The essential features in the history of a young lady presenting with symptoms for 19 years with shortness of breath since the age of 10 years which has worsened recently with the onset of palpitations at rest.

The possibilities to be considered at this point would be:

  • Valvular heart disease likely stenotic, with a recent arrhythmia-like atrial fibrillation (AF)
  • Acyanotic congenital heart disease (like atrial septal defect [ASD]) with recent arrhythmia like AF.


The onset of dyspnea in childhood brings up the possibility of congenital heart disease, valvular heart disease, and various types of cardiomyopathies. The disease has been very slowly progressive and has worsened only recently with a possible arrhythmic event since there are palpitations present at rest for 5 months. There is no evidence of a large left to right shunt in childhood, and stenotic lesions which present in childhood are rapidly progressive downhill. Cardiomyopathies in childhood would be infiltrative or dilated cardiomyopathies which would either have a rapid downhill course or recover, a bimodal course cannot be explained by a cardiomyopathy. This would leave the possibility of a moderate shunt like an ASD, which has been worsened by an atrial arrhythmia or a rheumatic valvular lesion like mitral stenosis now worsened by AF.

In a series of 128 adult patients with ASD, [1] with ages from 18 to 67, symptoms were mild and initially nonprogressive and 14% developed severe pulmonary hypertension between 20 and 40 years of age. Seventy-nine percent had dyspnea, but most were not disabled. Ten had AF, all of whom were above 40 years of age.

Patients with mitral stenosis have a bimodal presentation. In the series of juvenile mitral stenosis by Roy et al., 6% of the patients had AF, 78% had significant symptoms, and in two-thirds the pulmonary artery pressure was high. Seventy percent of the patients with a history of rheumatic fever had their first symptoms within 5 years of the first episode of rheumatic fever. These patients were sicker and presented early for intervention. Those who are not so sick can linger on and present later like our patient. [2]

The course of chronic mitral stenosis, from inception during rheumatic fever to the point where an intervention becomes necessary, usually spans a period of several decades if they do not present like juvenile mitral stenosis. Bland and Jones in their 20 years follow-up in children with acute rheumatic fever described that the development of mitral stenosis took longer than a decade. In the series by Wood, latent period was an average of 16 years, mean age of onset of 12 years, and age at appearance of symptoms 31 years. Thus, patients with mitral stenosis develop symptoms in the fourth or fifth decade of life. In half, symptoms develop gradually, and in the other half abruptly often precipitated by a complication such as AF. When symptomatic, some can be controlled by medical management, others may deteriorate rapidly and an intervention becomes necessary. [3],[4],[5]

Overall, the history is suggestive of a valvular heart disease since childhood, likely a left-sided lesion with recent worsening due to an arrhythmia. A long history of dyspnea suggests mitral stenosis with AF as the first differential diagnosis.

Prolonged shortness of breath which is slowly progressive with no associated palpitation and toward the later stages gets worsened with palpitations is suggestive of a stenotic lesion like mitral stenosis. Mitral regurgitation also has progressive dyspnea, but the clue comes from palpitations which come relatively early in the history and persist and increase as the symptoms increase. These are absent here. Moreover, absence of palpitation would point against regurgitant lesions at the aortic valve.


  Cardiovascular Examination Top


  • She is a thinly built and poorly nourished lady with a body mass index of 14.02 kg/m 2
  • The pulse was 92 bpm, irregularly irregular, with no radio femoral delay and all pulses being present
  • The blood pressure was 122/80 in the right arm. The pulse deficit was 10 bpm
  • Jugular venous pressure (JVP) is raised 08 cm above the angle of Louis with absent "a" wave, and presence of "cv" wave
  • There was no pedal edema. There is a Grade II parasternal heave. Second sound not palpable
  • S1 normal, S2 normally split, with a load P2, an opening snap was present
  • There was a precordial bulge. The apical impulse was palpated in the 5 th Lt intercostal space, 01 cm medial to left mid-clavicular line. Apex was localized, ill sustained
  • There was a systolic thrill present at the apex and lower left sternal border. Left parasternal heave is present Grade II/III.


Auscultation

  • There was a pansystolic blowing systolic murmur, Grade IV/VI, radiating to the axilla
  • An additional mid-diastolic murmur (MDM) was present which was low-pitched, rumbling nonradiating, best heard in lateral position with breath held in expiration, with presystolic extenuation
  • At the lower left sternal border, there was a Grade III/VI pan systolic murmur rough in character increased in inspiration.


Other systemic examination was unremarkable. There was no hepatomegaly or upper abdominal tenderness. The optic fundus was normal.

Examiners

Can you highlight the salient positive or negative features in the examination which will aid in your diagnosis.

Positive diagnostic features:

  • Irregularly irregular pulse
  • Raised JVP with absent a wave and presence of cv waves
  • Left ventricular (LV) type of apex with a pansystolic murmur (PSM) and an MDM at the apex. PSM at lower left sternal border
  • Loud S2 and presence of LPSH.


Diagnosis:

Rheumatic heart disease with severe MS, mod mitral regurgitation (MR), and moderate TR in AF, New York Heart Association (NYHA) Class IV with mod Pulmonary Arterial Hypertension (PAH) with severe PVH with congestive cardiac failure with no evidence of Infective endocarditis (IE) or acute rheumatic fever.

What are the causes of loud S1 in MR?

  • Tachycardia
  • Mitral valve plasty (MVP) with MR
  • Acute rheumatic carditis
  • Anemia
  • Thin chest wall.


Examiners

What are the causes of sudden deterioration in a patient with valvular heart disease.

  • New onset AF
  • Infective endocarditis
  • Rheumatic carditis
  • Pregnancy
  • Anemia
  • Infection/sepsis
  • Thyrotoxicosis.


Examiners

How do you decide which is the dominant lesion: MS or MR?

MR dominance is suggested by an LV hyperdynamic apex with an LV S3 while MS dominance is suggested by a normal apex with severe PAH and an opening snap and no S3.

What is the incidence of AF in MS.

  • Is according to the age of the patient, LA diameter and is different in different series. Less than thirty years of age - 10%, >50 years of age - 50%
  • LA <40 mm - 5%, LA >40 mm - 50%
  • In Various series, <30 years - 17% and >50 years - 80% patients had AF.


What are the conditions that can mimic Mitral stenosis clinically?

  • LA myxoma
  • Ball valve thrombus in the left atrium (usually associated with MS)
  • Infective endocarditis with large vegetations
  • Cortriatriatum
  • Diastolic flow murmur across normal valve in VSD, PDA, severe MR
  • Carey Coomb's murmur of mitral valvulitis.


Under which conditions does a patient with mitral stenosis feel better with regards to dyspnea spontaneously?

In a case of mitral stenosis, dyspnea improves spontaneously once the patient develops.

  • Organic tricuspid valve disease (TS)
  • Development of precapillary stenosis (sometimes called "secondary MS")
  • Development of right heart failure secondary to PAH.


What are the features that help in identifying the severity of MR?

  • Wide splitting of S2, loud P2
  • Loud palpable S3
  • Presence of MDM at apex (due to increased flow across mitral valve)
  • Brisk carotid pulse
  • Presence of systolic thrill
  • Presence of AF
  • Downwardly displaced apex beat with "sustained" apical impulse
  • Longer and harsher murmur
  • Early or late systolic parasternal impulse.


How would you differentiate etiology of MR from auscultation?

  • Wide splitting S2 - chronic severe MR likely rheumatic
  • Normally splitting S2 with loud S1 (systolic honk) - MVP
  • Paradoxical splitting S2, cooling quality of murmur-papillary muscle dysfunction (LV dysfunction).


What is the natural history of MR?

  • 5 years survival in symptomatic patients who refused surgery was 30% only
  • In MR due to flail mitral leaflet, annual mortality was 6.3%.



  Let Us Now Look at the Investigations Top


Investigations

The electrocardiogram [Figure 1] showed AF with a controlled ventricular rate, with no evidence of LV hypertrophy or pulmonary hypertension. The Chest X-ray [Figure 2]. showed evidence of pulmonary venous hypertension and pulmonary edema and pulmonary artery hypertension along with evidence of left atrial and right atrial enlargement. There was no evidence of significant enlargement of the left ventricle.
Figure 1: Electrocardiogram. Showing atrial fibrillation with controlled ventricular rate and normal axis with no left ventricular hypertrophy.

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Figure 2: Chest X-ray. Showing left atrial and right atrial enlargement with pulmonary edema with pulmonary venous and arterial hypertension. Minimum left ventricular dilatation.

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The echocardiogram [Figure 3],[Figure 4] and [Figure 5] showed a normal-sized left ventricle with normal systolic function. There was severe mitral stenosis with moderate MR. There was also evidence of the organic involvement of the tricuspid valve with tricuspid annular dilatation, tricuspid regurgitation along with some pulmonary hypertension.
Figure 3: (a) Showing mitral stenosis turbulence across the mitral valve. (b) Showing mitral regurgitation (at least moderate). (c) Shows a mean transmitral gradient of about 23 mmHg.

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Figure 4: M mode showing the dimensions. Left atrium is dilated. Left ventricle is not dilated and left ventricular function = 68.3%. No evidence of LVH in figure 4b.

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Figure 5: Showing echo across the tricuspid valve. (a) Showing tricuspid annular dilatation (black arrow). (b) Shows tricuspid regurgitation. (c) Showing tricuspid regurgitation Doppler with a gradient of 54 mmHg at the tricuspid valve. TV annulus 46 mm.

Click here to view


The final diagnosis was:

  • Severe mitral stenosis with moderate MR
  • Organic tricuspid valve disease with tricuspid regurgitation
  • Severe pulmonary venous and arterial hypertension
  • AF with a controlled ventricular rate
  • No rheumatic activity or infective endocarditis
  • NYHA Class III.


The plan is:

Mitral valve replacement with tricuspid valve repair.


  What is the Plan for Surgery Top


For the mitral valve, the patient has both MR and mitral stenosis. Approximately 25% of all patients with rheumatic heart disease (RHD) have pure MS, and an additional 40% have combined MS and MR. The decision in mixed valve lesions is based on the dominant valve lesion. If the LV is near normal in size, then mitral stenosis is dominant, and if LV is very dilated then MR is dominant. In this case, the dominant lesion is the mitral stenosis. Since mitral stenosis is severe, intervention is justified, but the regurgitation precludes any closed valvotomy procedure and therefore the only procedure for the mitral valve is a mitral valve replacement (MVR) with a prosthetic valve. The valve at her age would a metallic valve unless she has to complete her family in which case a bioprosthetic valve may be considered.

For the tricuspid valve, tricuspid valve annuloplasty should be done with the insertion of a Carpentier ring.


  What are the Results of Mitral Valve Interventions? Top


At 7 years after balloon mitral valvotomy (BMV), 50-69% of patients remain free of cardiovascular events and up to 90% of patients remain free of re-intervention. Complications of BMV include severe MR (3%), thromboembolism (3%), and residual ASD with significant shunting (<5%). Mortality with the procedure is lower than 1% in experienced hands. [6],[7]

In a surgical series of valve replacements, [8] five hundred and seventy-three patients underwent mitral valve surgery for rheumatic disease from 1978 to 1995. Mean age was 54 ± 14 years mitral stenosis was present in 53%, regurgitation in 15%, and both in 32%. Valve repair was performed in 25%, bioprosthetic replacement was performed in 28%, and a mechanical valve was placed in 47%. The operative mortality rate was 4.2%. There was a 10-year survival rate of only 73% ± 6.0% at 10 years after implantation of a mechanical mitral prosthesis. This late mortality is attributed largely to thromboembolic events and bleeding complications of long-term anticoagulation. In the carefully selected patients in whom valve repair was performed an 88% 10-year survival rate compared with a 70% rate for bioprostheses and a 73% rate for mechanical valves.

What are the predictors of excellent results of MVR in MR?

  • Excellent prognosis- LV end systolic dimension <45-52 mm, left ventricular ejection fraction (LVEF) =50-60%
  • Age <60 years
  • NHYA Class II
  • CI >2 L/min/m 2
  • LVEDP <12 mmHg
  • RHD etiology has a better prognosis than ischemic MR (75% vs. 40% 5 years survival).


Examiners

What are the indications of MVR in asymptomatic patients with MR?

  • LVESD >45 mm or
  • LVEF <60% or
  • Evidence of pulmonary hypertension or new onset AF
  • LA size >45-50 mm is taken as supportive evidence in severe MR by some surgeons.


Examiners

What are the indications of tricuspid valve repair/replacement.

The new European guidelines recommend surgery on the tricuspid valve of these patients if TR is severe or provided the annulus is ≥40 mm or ≥21 mm/m΂, independently of the regurgitation.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Craig RJ, Selzer A. Natural history and prognosis of atrial septal defect. Circulation 1968;37:805-15.  Back to cited text no. 1
    
2.
Roy SB, Bhatia ML, Lazaro EJ, Ramalingaswami V. Juvenile mitral stenosis in India 1963. Natl Med J India 2011;24:248-53.  Back to cited text no. 2
    
3.
Wood P. An appreciation of mitral stenosis. Br Med J 1954;1:1051-113.  Back to cited text no. 3
    
4.
Rowe JC, Bland EF, Sprague HB, White PD. The course of mitral stenosis without surgery: Ten- and twenty-year perspectives. Ann Intern Med 1960;52:741-9.  Back to cited text no. 4
    
5.
Bland EF, Duckett Jones T. Rheumatic fever and rheumatic heart disease; a twenty year report on 1000 patients followed since childhood. Circulation 1951;4:836-43.  Back to cited text no. 5
    
6.
Ben Farhat M, Ayari M, Maatouk F, Betbout F, Gamra H, Jarra M, et al. Percutaneous balloon versus surgical closed and open mitral commissurotomy: Seven-year follow-up results of a randomized trial. Circulation 1998;97:245-50.  Back to cited text no. 6
    
7.
Hernandez R, Bañuelos C, Alfonso F, Goicolea J, Fernández-Ortiz A, Escaned J, et al. Long-term clinical and echocardiographic follow-up after percutaneous mitral valvuloplasty with the Inoue balloon. Circulation 1999;99:1580-6.  Back to cited text no. 7
    
8.
Yau TM, El-Ghoneimi YA, Armstrong S, Ivanov J, David TE. Mitral valve repair and replacement for rheumatic disease. J Thorac Cardiovasc Surg 2000;119:53-60.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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