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Right Ventricular Failure
Right Ventricular Failure
Introduction
Many a time the right ventricle (RV) is regarded as the 'younger brother' of the left ventricle (LV)
and is treated as a less important member of the contractile apparatus. This view stems from the
concept that the RV functions rather as a passive conduit and its importance is not great as it pumps
blood to only one organ, the lungs. However, the circulatory system is a closed one, and both
ventricles are interdependent working together in an orchestrated complex pattern in health and
disease. The failure of one ventricle deleteriously affects the performance of the other. This review
will deal with the causes of right ventricular failure and its diagnosis, leaving the management to a
second part of this series.
Background
Prevalence
Right heart failure as the primary presentation of acute decompensated HF and cause of
hospitalisation accounted for 2.2% of HF admissions in the CHARITEM registry; [2] however, it
was present as secondary to acute LV failure in more than one-fifth of the cases.
In our Egyptian Heart Failure-LT registry, 4.5% of patients with acute heart failure presented with
RHF as opposed to 3% in other ESC regions. [3] This could be attributed to the even higher
incidence of rheumatic heart disease. In support of this, Hassanein et al. reported that the incidence of
valvular heart disease was more than double than in the other ESC regions (17.5% of our cohort
versus 8%) in the same registry. [3] Rheumatic fever affects the mitral valve mainly in the form of
stenosis, but also mitral regurgitation or a combination of both. Neglected mitral valve disease results
in pulmonary hypertension, severe functional tricuspid regurgitation and right-sided heart failure. In
addition, rheumatic heart disease not infrequently causes organic tricuspid valve disease (stenosis,
regurgitation or a combination of both). Decades ago, infection by S. Mansoni caused pulmonary
hypertension in Upper Egypt, but the eradication programmes for schistosomiasis have resulted in
this problem now being a rare occurrence. It is still endemic in sub-Saharan Africa.
PH types 1, 3, 4 and most of 5 in the WHO classification are pre-capillary; all are characterised by
low or normal wedge pressure. The second most common cause of PH is secondary to lung disease.
The commonest lung diseases are obstructive airway disease followed by lung fibrosis. Another
important cause which is frequently overlooked is obstructive sleep apnoea (OSA). PH is present in
17-53% of individuals with OSA [5]. Lung diseases cause pulmonary hypertension via hypoxia
which causes polycythaemia, vasoconstriction and vascular remodelling, in addition to damage of
lung parenchyma with loss of vascular bed.
A particular type of PH results from acute pulmonary embolism, and can result in acute right heart
failure as the RV fails to maintain blood flow past an obstructing large embolus. Recurrent showers
of smaller pulmonary emboli can end in chronic thromboembolic pulmonary hypertension (CTEPH).
Here emboli do not completely resolve, but they partially recanalise and are endothelialised, resulting
in pulmonary artery obstruction.
All congenital heart diseases with increased pulmonary blood flow, mainly left-to-right shunts, can
lead to PH. The development of PH depends on the duration of exposure and its magnitude, i.e.,
ventricular septal defect and patent ductus arteriosus (post-tricuspid defects) patients tend to develop
PH earlier than atrial septal defect patients (pre-tricuspid).
Pulmonary hypertension type 1 is idiopathic or secondary to connective tissue. Idiopathic PAH
affects mostly females. It is thought to be caused by an imbalance of vasodilator NO pathway and
vasoconstriction endothelin-1 pathway. It is characterised by increased pulmonary vascular resistance
due to remodelling and occlusion of the pulmonary arterioles.
Less commonly, RV failure could result from direct affection of myocardial disease by myocarditis,
cardiomyopathy, ischaemia, or arrhythmia. Right ventricular infarction complicates 30–50% of
inferior myocardial infarction and it is usually caused by occlusion of the proximal right coronary
artery. Compared with the left ventricle, the right ventricle is more resilient in the face of ischaemia.
This is due to less myocardial oxygen demand, coronary perfusion occurring throughout the cardiac
cycle, and a dual blood supply - the left anterior descending artery supplies the anterior two-thirds of
the septum. So, in the majority of cases, the RV recovers within a few days. However, during the
initial presentation profound hypotension and shock may be present.
The tricuspid valve is organically affected in rheumatic heart disease, in infective endocarditis in IV
drug addicts, or by trauma caused by pacemaker electrodes during implantation or retrieval. Ebstein’s
anomaly frequently presents as right heart failure in children or in early adulthood.
Gradual accumulation of fluid in the pericardial sac can compress the thin-walled RV and prevent its
filling, presenting as RHF. Constrictive pericarditis is one of these diagnoses which can be easily
missed. It is caused by fibrosis and calcification of the encasing pericardium, restricting diastolic
filling of the ventricles. The commonest cause used to be prior tuberculosis infection, but nowadays
it is mostly secondary to chest radiotherapy or previous cardiac surgery.
Clinical diagnosis
Symptoms of right heart failure are mainly due to systemic venous congestion and/or low cardiac
output. This includes exertional dyspnoea, fatigue, dizziness, ankle swelling, epigastric fullness and
right upper abdominal discomfort or pain.
In taking past medical history it is very important to inquire about the presence of coronary artery
disease, emphysema/chronic bronchitis, history of deep venous thrombosis, recurrent abortions,
autoimmune diseases – especially scleroderma and systemic lupus erythematosus (SLE) – and
infections, e.g. HIV, tuberculosis and schistosomiasis. Family history of PAH can be present as some
cases of PAH can have a familial occurrence.
Signs: raised jugular venous pulse (JVP), left parasternal lift, an accentuated second pulmonary
sound, right ventricular gallop, usually a pansystolic murmur over the tricuspid area which increases
with inspiration, and sometimes diastolic murmur of pulmonary insufficiency; also, an enlarged
tender liver, ascites frequently present as well as ankle oedema.
It is worth mentioning a few points to highlight the importance of raised JVP as a clinical sign. It is a
specific sign of right heart failure and reflects raised right atrial pressure. It correlates well with
raised left heart filling pressure in LV failure. Raised JVP is a prognostic marker. Analysis of the
SOLVD study has shown that it correlates with mortality and a risk of heart failure hospitalisation in
LVF. [6] Kussmaul's sign, which is an increase of JVP on inspiration, can help in pointing to the
cause of RHF. It is caused by impaired RV diastolic compliance with increased venous return, as
seen in constrictive pericarditis and RV infarction.
Right ventricular infarction should be suspected in the context of inferior MI by the triad of raised
JVP, hypotension and clear lung fields.
Biochemical markers
Systemic venous congestion affects the liver and kidney and results in derangement of their function.
Raised transaminases and bilirubin plus prolonged prothrombin time are common in right HF and
reflect poor prognosis. [11] Raised renal chemistry is frequently noted and may improve with
diuretics.
There are no specific biomarkers for right heart failure, but raised BNP and troponins reflect stress
and injury in different RHF scenarios. Their rise reflects the severity of the condition and portends
poor prognosis. For example, Krüger [12] has noted that BNP is elevated in acute pulmonary
embolism complicated by RV dysfunction, but is within normal range when RV function is
preserved. Patients with pulmonary embolism and plasma lactate level >2 mmol/L are at high risk of
death and adverse outcome. [13]
Conclusion
Even though right ventricular failure does not take centre stage in the field of heart failure research
and clinical trials, it is actually the final pathway of left-sided heart failure. Pulmonary hypertension
is the commonest cause of right heart failure. Other causes are RV myocarditis, genetic
cardiomyopathy, ischaemia ,as well as pericardial disease. Due to the unusual anatomy of RV,
assessment of its function is a challenge. However, technical advances, especially in
echocardiography and cardiac MRI, are helping to evaluate RV function and volumes, as well as
measurement of pulmonary artery pressure. Careful history-taking, clinical examination and the
targeted use of investigations can elucidate the underlying pathology.
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