What is the pathology in Ebstein’s anomaly ?
The tricuspid leaflets are abnormal and are displaced into the body of the right ventricle.
The septal leaflet
is variably deficient or even absent.
The posterior leaflet is also variably deficient and there is a large
‘sail-like’ anterior leaflet that is the hallmark of this condition.
The abnormally located tricuspid orifice
results in a part of the right ventricle lying between the atrioventricular ring and the origin of the valve,
which is continuous with the right atrial chamber.
This proximal segment is known as the ‘atrialized’
portion of the right ventricle.
About 50% of the patients have either a patent foramen ovale or a secundum ASD, and 25% have
one or more accessory atrioventricular conduction pathways.
The anomaly is said to be associated with
maternal lithium ingestion.
What treatment is available for primary pulmonary hypertension ?
• Diuretics are useful in reducing excessive preload in patients with right heart failure, particularly when hepatic congestion and ascites are present.
• Oral anticoagulants: warfarin is the anticoagulant of choice, in doses adjusted to achieve an INR of
approximately 2.0. Anticoagulants nearly double the 3-year survival rate (Circulation 1984; 70: 580-7).
* Calcium channel blockers: nifedipine, diltiazem. Patients who respond to calcium channel blockers
have a 5-year survival rate of 95% (N Engl J Med 1992; 327: 76-81).
• Intravenous epoprostenol (formerly prostacyclin or prostaglandin I2), which is a potent short-acting
vasodilator and inhibitor of platelet aggregation that is produced by the vascular endothelium (N Engl
J Med 1996; 334: 296-301; N Engl J Med 1998; 338: 273-7).
• Atrial septostomy: the creation of a right-to-left shunt by blade-balloon atrial septostomy has been
reported to improve forward output and alleviate right-sided heart failure by providing blood with a
low-resistance channel, thereby decompressing the right atrium and improving filling of the left side of
the heart (Circulation 1995; 91: 2028-35).
• Lung transplantation and combined heart-lung transplantation: survival rates after the two
procedures are similar. Even markedly depressed right ventricular function improves considerably
with single- or double-lung transplantation.
• Possible future drugs: (a) UT-15, a prostaglandin 12 analogue, has been shown to have sustained
and favourable effects in patients when administered sub-cutaneously (Circulation 2000; 102(18):
11-101); (b) Sitaxsentan, an oral selective endothelin-A receptor blocker, has been shown to produce
sustained improve-ments in pulmonary artery pressure (Circulation 2000; 101(25): 2922-7).
Graham Steell (1851-1942), assistant physician to the Manchester Royal Infirmary, described the murmur in a paper titled The murmur of high pressure in the pulmonary artery (Med Chron (Manchester) 1888; 9: 182-8).
What is the prognosis in pulmonary hypertension ?
The prognosis is poor: median survival is approximately 3 years from the time of diagnosis, with about
one third of patients surviving for 5 years.
Death usually occurs suddenly, presumably from arrhythmias
or right ventricular infarction.
What are the theories for the cause of primary pulmonary hypertension ?
• Excess endothelial production of the vasoconstrictor thromboxane relative to dilator prostaglandins
such as prostacyclin.
• Excess endothelin-I levels relative to nitric oxide. Inhaled nitric oxide and endothelin-1 antagonists
reduce pulmonary hypertension.
• Excessive thrombosis in situ due to increased platelet activation, plasminogen activator inhibitor
levels and decreased thrombomodulin.
• Increased serotonin levels.
• Inhibition or downregulation of potassium (Kv) channels in pulmonary artery smooth muscle cells
• Activation of elastase and matrix metalloprotease enhances production of mitogens.
• Monoclonal proliferation of endothelial cells.
What are the pathological features of primary pulmonary hypertension ?
They are those of plexogenic pulmonary arteriopathy (which also occurs in post-tricuspid left-to-right
atrial shunts such as VSD or PDA, and collagen vascular diseases), characterized by medial hypertrophy
and concentric intimal fibrosis of the pulmonary arteries with complex plexiform lesions.
Others have no
plexiform lesions or concentric intimal fibrosis, but rather have recanalized thrombotic small pulmonary
arteries which are said to be the result of small thrombi or recurrent emboli.
The least common
histological pattern is veno-occlusive disease.
What are the signs of pulmonary hypertension ?
• Large ‘a’ waves in JVP.
. Left parasternal heave.
• Loud or palpable P2.
• Ejection click in the pulmonary area.
• Early diastolic murmur (Graham Steell murmur) due to pulmonary regurgitation.
What are the functions of pericardium ?
• The pericardium protects and lubricates the heart.
• It contributes to the diastolic coupling of the left and right ventricles – an effect that is important in
cardiac tamponade and constrictive pericarditis.
W. Dressier (1890-1969), US physician educated in Vienna. He worked at the I
Manimoides Hospital, Brooklyn, New York.
What do you know about postcardiotomy syndrome ?
It occurs in about 5% of patients who have cardiac surgery, with symptoms of pericarditis from three
weeks to six months after surgery. It is initially treated with NSAIDs and systemic steroids in refractory
Pericardiectomy is rarely required. It is said to result from an autoimmune response and is most
likely to be related to surgical trauma and irritation of blood products in the mediastinum and pericardium.
What is Dressler’s syndrome ?
Dressler’s syndrome is characterized by persistent pyrexia, pericarditis and pleurisy.
It was first described
in 1956 when Dressier recognized that post-myocardial infarction chest pain is not caused by coronary
It usually occurs 2-3 weeks after myocardial infarction and is considered to be of
autoimmune aetiology; it responds to NSAIDs.
What do you know about the transient constrictive phase of acute
About 10% of the patients with acute pericarditis have a transient constrictive phase which may last 2-3
months before it gradually resolves, either spontaneously or with treatment with anti-inflammatory drugs.
These patients usually have a mod-erate amount of pericardial effusion and, as the effusion resolves, the
pericardium remains thickened, inflamed and non-compliant resulting in constrictive haemo-dynamics.
Clinical features include shortness of breath, raised jugular venous pressure, peripheral oedema and
Constrictive haemodynamics can be documented by Doppler echocardiography and resolution of
constrictive physiology can be serially followed by this technique.