• Users Online: 280
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
REVIEW ARTICLE
Year : 2020  |  Volume : 6  |  Issue : 1  |  Page : 18-22

Multivalvular heart disease: Case discussion


Department of Cardiology, AIIMS, New Delhi, India

Date of Submission08-Mar-2020
Date of Acceptance20-Mar-2020
Date of Web Publication17-Apr-2020

Correspondence Address:
Ram Manohar Talupula
Senior Resident, Department of Cardiology, AIIMS, New Delhi
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpcs.jpcs_13_20

Rights and Permissions
  Abstract 


Here we report a bedside case discussion of a young patient with multivalvular heart disease who presented with dyspnea, palpitations, abdomen distension, yellow eyes, and pedal edema. There were an irregular pulse, engorged neck veins, systolic murmurs in the apex and parasternal areas, and a diastolic parasternal murmur. The diagnosis of multivalvular disease is discussed based on the history and clinical findings. The help of the electrocardiogram, X-ray, and echocardiography in arriving at the diagnosis is also discussed in this bedside case discussion for students.

Keywords: Abdominal distension, multivalvular, yellowish discoloration


How to cite this article:
Talupula RM, Rajesh R A. Multivalvular heart disease: Case discussion. J Pract Cardiovasc Sci 2020;6:18-22

How to cite this URL:
Talupula RM, Rajesh R A. Multivalvular heart disease: Case discussion. J Pract Cardiovasc Sci [serial online] 2020 [cited 2020 May 27];6:18-22. Available from: http://www.j-pcs.org/text.asp?2020/6/1/18/282799




  Presenting Complaints Top


A 22-year-old female, resident of Bihar, who is studying BA, presented with the chief complaints of:

  • Shortness of breath for 2 years
  • Palpitations for 2 years
  • Abdominal distension for 1½ years
  • Yellowish discoloration of eyes for 1½ years
  • Swelling of both feet for 4 months.



  History of Present Illness Top


The patient was apparently well till about 2 years ago when she initially started feeling breathless after walking for about 1 km and needed to take rest to continue further. This gradually progressed in the next 2 months that she was unable to walk for more than 10–15 steps without feeling breathless. This was associated with orthopnea.

At around the same time, the patient also developed palpitations on exertion. Palpitations were of gradual onset and gradual offset. There were no palpitations at rest. There was no history of increased urination following the episode of palpitations.

The patient also developed abdominal distension 1½ years ago. Initially, she had complaints of early satiety of food, abdominal discomfort, and fullness. This was associated with nausea and vomiting on consumption of food. Then, she noticed gradually progressive abdominal distension which progressed over the next 6 months and then resolved after she was given some medications for the same.

The abdominal distension was associated with yellowish discoloration of the eyes, which were noticed by the patient and the family members. The yellowish discoloration of the eyes is currently persisting. There was no history of itching or clay-colored stools, blood in vomitus, and black tarry stools.

She then noticed swelling in both her feet 4 months ago which gradually progressed in the next 2 weeks to involve both her legs and her entire body with facial swelling. This has resolved since the patient was given some medications which increased her urine output.

There is no history of chest pain and syncope.

No history of fever, weight loss, small joint pain, chronic diarrhea, and injection drug abuse was noted.


  Past History Top


At 5 years of age, she had a history of swelling associated with pain in her large joints on both sides. This lasted for about 6 months. She was then evaluated for the same by a doctor who told her family that she had a cardiac problem and was advised to take injections once every 3 weeks and was also advised oral medication.


  Family History Top


  • No similar complaints in her family members.



  Personal History Top


She consumes a mixed diet. Currently, her sleep and appetite are normal. She is unmarried and her last menstrual period was 4 months ago. She has been having irregular periods since the last 2 years.


  Treatment History Top


Since 5 years of age, she was taking injections once every 3 weeks for about 2 years, and after that, she was irregular with taking injections.

Since the last 2 years, she has been taking medications for symptomatic relief from several general practitioners.


  System Review Top


  • No history of cough, expectoration, wheeze
  • No history of abdominal pain, diarrhea
  • No history of involuntary movements, weakness involving the limbs, deviation of face, sudden obscuration of vision.



  Summary Top


A 22-year-old female with a history suggestive of acute rheumatic fever presented with insidious onset, gradually progressive shortness of breath, and palpitations on exertion for 2 years followed by features of right heart failure (HF) for the last 1½ years.

Question

Before examining the patient, what were your differential diagnoses in this patient?

This is a case of early right HF after left HF. Had there been only systemic congestion, with no dyspnea, the pathology might be localized to the right-sided chambers.

The onset of dyspnea at this age brings up the possibility of congenital heart disease, valvular heart disease, and various types of cardiomyopathies. The differential diagnosis of such a clinical scenario would include:

  • Rheumatic heart disease (RHD) with left and right valve involvement
  • Restrictive cardiomyopathy (RCMP) with valve involvement
  • Dilated cardiomyopathy (DCMP)
  • Ventricular septal defect + AR +pulmonary arterial hypertension (PAH)
  • Lutembacher syndrome.


Question

What are the causes of early and out-of-proportion congestive HF in valvular heart disease?

  • Organic tricuspid valve (TV) disease
  • Lutembacher
  • Ruptured sinus of Valsalva aneurysms
  • Endomyocardial fibrosis/chronic constrictive pericarditis (EMF/CCP)
  • Other precipitating factors for HF.



  Examination Top


She was conscious and oriented to time, place, and person.

Pulse was 74 beats per minute, irregularly irregular, had normal volume, no particular character, all peripheral pulses palpable, no R–R or R–F delay, and no vessel wall thickening.

Blood pressure in the right arm was 104/72 mmHg and in the left arm was108/72 mmHg while in the right lower limb was 110/70 mmHg and in the left lower limb was 114/72 mmHg. There was no orthostatic hypotension.

Respiratory rate was 22 breaths per minute, thoracoabdominal.

She was afebrile.

Jugular venous pressure (JVP) elevated 10 cm above the sternal angle with the head end elevated at 45°, A wave absent, CV wave present, and prolonged Y descent.

General examination

  • Height –158 cm, weight –40 kg, body mass index –16 kg/m2
  • She was thin built. Pallor and icterus were present
  • Pitting pedal edema/sacral edema +
  • No clubbing, cyanosis
  • No peripheral signs of infective endocarditis
  • No features of Marfan syndrome seen
  • No peripheral signs of aortic regurgitation


Systemic examination

Cardiovascular examination

Inspection

  • Apical impulse is seen lateral and inferior to the left nipple
  • No precordial bulge
  • No dilated veins or other localized pulsating. Chest is bilaterally symmetrical and moving equally with respiration. No kyphoscoliosis. No scars or sinuses.


Palpation

Findings from inspection were confirmed. Apical impulse is located 2 cm lateral to the mid-clavicular line in the 5th intercostal space which is hyperdynamic in character.

There is a palpable second heart sound and Grade I left parasternal heave.

A systolic thrill is felt over the apex, tricuspid, and the aortic area.

Auscultation

S1 is soft.

S2 is narrow split and loud with a loud P2 component.

There is no S3 or S4.

There are no additional sounds.

There is a high-pitched, blowing pansystolic murmur of Grades IV/VI intensity in the mitral area radiating to the axilla, which is better heard in the left lateral position with breath held in expiration. Standing or Valsalva maneuver did not change the intensity of the murmur.

There is also a low-pitched rough and rumbling mid-diastolic murmur in the mitral area, with no radiation or presystolic accentuation (patient in atrial fibrillation [AF]). It is best heard in the left lateral position with breath held in expiration. There is no opening snap.

Over the tricuspid area, there is a high-pitched prolonged pansystolic murmur of Grade IV/VI intensity which increases on inspiration and better heard in sitting position. There is no radiation of the murmur. A localized mid-diastolic murmur is also heard over the tricuspid area.

In the aortic area, there is a low-pitched ejection systolic murmur of Grade IV/VI intensity, radiating to the carotids, which is best heard with the patient sitting up and leaning forward.

There is also an early diastolic murmur of short duration in the neo-aortic area best heard in sitting and leaning forward position with no radiation.

Gastrointestinal system

Abdomen was soft, nontender, hepatomegaly with pulsatile liver present with the liver margin palpable 6 cm below the right costal margin. Liver span was 16 cm. There was no splenomegaly.

Respiratory system

  • Normal chest expansion.
  • Bilateral basal fine crepitation heard.


Nervous system

  • Normal motor and sensory system examination. Chest X-ray and ECG of the patient are shown in the [Figure 1] and [Figure 2] respectively.
Figure 1: Chest radiograph showing cardiomegaly with a computed tomography ratio of 0.8 with biatrial enlargement and right ventricular type of apex. There is also pulmonary venous hypertension.

Click here to view
Figure 2: Electrocardiogram showing coarse atrial fibrillation with digoxin effect in inferior leads and non-specific ST-T changes in V4–V6.

Click here to view



  Clinical Diagnosis Top


  • RHD
  • Severe mitral regurgitation (MR), moderate mitral stenosis (MS)
  • Severe AS, moderate AR
  • Organic TV disease (severe tricuspid regurgitation (TR), tricuspid stenosis [TS] +)
  • Moderate PAH with severe pulmonary venous hypertension
  • With congestive cardiac failure
  • Normal biventricular function
  • NYHA III, AF with controlled ventricular rate
  • No evidence of infective endocarditis or acute rheumatic activity.


Question

What are the causes of multivalvular heart disease?

  • RHD
  • Congenital diseases
  • Inherited diseases – Marfan syndrome
  • Inflammatory – Immunological conditions
  • Endocardial disorders
  • Myocardial dysfunction
  • Disorders of other organs – Carcinoid
  • Aging
  • Postinterventional valvular disease
  • Drugs and physical agents.


Question

In a patient with severe MR, how do you assess whether there is associated MS?

The presence of the following would help us decide on examination whether there is

  • MS in cases of severe MR
  • Thrill
  • Prolonged medical decision-making
  • Opening snap
  • Loud S1
  • Severe PAH.


Question

In a patient with both MS and MR, how do you assess which is severe? [Table 1]
Table 1: Clinical clues to differentiate a more dominant lesion in a mixed lesion

Click here to view


Question

Clinically, when do you suspect organic TV disease?

TS easily escapes detection. Presence of disproportionate fatigue and CHF and less paroxysmal nocturnal dyspnea (PND) or orthopnea can help in suspecting organic TV disease. JVP is the key in such cases. A giant A wave with slow Y descent is diagnostic. Presence of murmur of TS also helps.

Question

What are the typical characteristics of a TS murmur?

  • Remarkable increase in the intensity of the murmur with inspiration so much that if this feature is absent diagnosis of TS is highly unlikely
  • Tricuspid diastolic murmurs are earlier in diastole than their mitral counterparts, so they can often be mistaken for early diastolic murmur of AR or PR


Question

What are the causes of short or no murmur in TS?

  • Rheumatic TS with MS, severe PAH, elevated right ventricular end diastolic pressure (RVEDP)
  • Diuretic therapy
  • AF (absent presystolic murmur)
  • Ebstein's anomaly.


Question

What are the clinical findings which differentiate an organic TR from functional TR (low-pressure vs. high-pressure TR)? [Table 2]
Table 2: Differences in examination findings of a high pressure TR from a low pressure TR

Click here to view



  Investigations Top


Echocardiography

RHD, severe MR, moderate MS, organic TV disease with severe TR and TS, severe AS, moderate AR, moderate PAH, normal biventricular function, mild circumferential pericardial effusion, no clot or vegetation. Left atrium (LA) diameter was 54 mm. Left ventricular internal dimension was 24/38.

Question

How do you identify LA enlargement on chest X-ray based on carina?

  • Interbronchial angle: Normal mean 67°–77° (range 34°–109°) (angle between the central axis of right and left main bronchi)
  • Subcarinal angle: Normal mean 62°–73° (range 34°–90°) (angle of divergence of right and left main bronchi measured along their inferior margins).


On chest radiograph, a carinal angle of more than 90° is an acceptable predictor of LA enlargement.

Moreover, an LA dimension more than 50 mm can be correctly predicted if the carinal angle is more obtuse (100° or greater). The size of the LA can be measured as the distance from the middle of the right lateral border of the LA to the middle of the inner margin of the left main bronchus. This distance is usually less than 7 cm in the majority of normal subjects and is generally 7 cm or greater in 90% of MS patients.

Question

Cardiac causes of ascites precox?

Constrictive pericarditis, organic TV regurgitation, and right ventricular cardiomyopathy (e.g., EMF).

Question

What are the gastrointestinal manifestations of HF?

Common gastrointestinal manifestations of HF include anorexia, early satiety, and abdominal pain.

Patients with advanced HF may also have ascites, protein losing enteropathy, and cachexia.

The bidirectional relationship of the heart and the gut in HF has been called the cardiointestinal syndrome.[1]

Mechanisms

  1. These symptoms have historically been attributed to poor abdominal organ perfusion or edema
  2. Hemodynamic changes in the splanchnic circulation. The sympathetic nervous system activation in HF leads to a decrease in the capacitance of splanchnic veins. This causes a shift of fluid out of the splanchnic veins, which increases the effective circulating volume. The above shift increases the preload without increasing total body volume and aggravates the effects of sodium and water retention
  3. Altered gut morphology and function – increased gut permeability, alteration in microbial composition in the gut
  4. Cardiac cachexia – increased in the factors that promote protein and fat tissue degradation. Upregulation of hormonal factors promoting catabolism – norepinephrine, epinephrine, TNFα, and cortisol. Decreased anabolic response – reduced dehydroepiandrosterone, insulin, and testosterone levels
  5. Protein losing enteropathy – gut edema and increased central venous pressure leading to rupture of intestinal lacteals.


Question

What are the hepatic manifestations of HF?

Liver involvement in HF: Congestive hepatopathy (most common), hepatic fibrosis, hepatic cirrhosis, and ischemic hepatitis.[1]

Mechanisms

  1. Congestive hepatomegaly – right-sided volume overload and elevated central venous pressure
  2. Hepatic fibrosis - prolonged congestion with wound-healing response – usually takes years
  3. Hepatic cirrhosis – end-stage fibrosis with portal hypertension and synthetic dysfunction
  4. Ischemic hepatitis – acute hypotension, more likely with decreased cardiac output and concomitant liver congestion.


Question

What is the current recommendation and evidence on the route of administration of penicillin and its frequency for secondary prophylaxis of acute rheumatic fever?

Studies on the effectiveness of antibiotics in the secondary prevention of rheumatic fever have shown two important findings.

  1. The superiority of intramuscular over oral penicillin.[2]
  2. More frequent injections are more effective than injections every 4 weeks


The evidence is strong for injections every 2 weeks, with an almost 50% reduction in the risk for recurrence of rheumatic fever when compared with injections every 4 weeks. The evidence for injections every 3 weeks is less strong. Despite this evidence, the WHO recommends intervals of 3–4 weeks for the secondary prevention of rheumatic fever.[2],[3],[4]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Ethics clearance

Ethical clearance taken from the ethical committee for publishing the case discussion.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sundaram V, Fang JC. Gastrointestinal and liver issues in heart failure. Circulation 2016;133:1696-703.  Back to cited text no. 1
    
2.
Manyemba J, Mayosi BM. Intramuscular penicillin is more effective than oral penicillin in secondary prevention of rheumatic fever-a systematic review. S Afr Med J 2003;93:212-8.  Back to cited text no. 2
    
3.
Gerber MA, Baltimore RS, Eaton CB, Gewitz M, Rowley AH, Shulman ST, et al. Prevention of rheumatic fever and diagnosis and treatment of acute streptococcal pharyngitis: A scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: Endorsed by the American Academy of Pediatrics. Circulation 2009;119:1541-51.  Back to cited text no. 3
    
4.
WHO Technical Report Series No. 923. Rheumatic Fever and Rheumatic Heart Disease: Report of a WHO Expert Panel. Geneva: WHO; 2004.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
   Abstract
   Presenting Compl...
   History of Prese...
  Past History
  Family History
  Personal History
  Treatment History
  System Review
  Summary
  Examination
  Clinical Diagnosis
  Investigations
   References
   Article Figures
   Article Tables

 Article Access Statistics
    Viewed104    
    Printed2    
    Emailed0    
    PDF Downloaded44    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]