Questions on Eisenmenger’s syndrome
Definition
- What is Eisenmenger’s syndrome? A, hs
- What is Eisenmenger’s complex? hs
Epidemiology
- Q
Etiology and Pathophysiology
- What are the causes of Eisenmenger’s syndrome? hs
- What happens to the murmur of VSD as Eisenmenger’s complex develops? hs
Clinical manifestations
- What are the clinical features of Eisenmenger’s syndrome? A
Examinations
- Why is second heart sound single and loud? Hs
- How would you differentiate Eisenmenger’s syndrome from TOF clinically? hs
Investigations
- What investigations do you suggest in this case? a
Diagnosis
- Why do you think Eisenmenger’s syndrome? hs
- Why do you think VSD is the underlying cause? Hs
- Why is Eisenmenger’s syndrome not due to ASD in this patient? hs
- Why is Eisenmenger’s syndrome not due to PAD in this patient? hs
- What are your differential diagnoses? A, hs
- Why not CCF? A
- Why not this is a case of chronic cor-pulmonale? a
Treatment
- How to treat Eisenmenger’s syndrome? A, hs
- What is the treatment? a
Complications
- What are the complications of Eisenmenger’s syndrome? hs
- What are the causes of death in Eisenmenger’s syndrome? A, hs
Rimikri
SOLVES
What is Eisenmenger’s syndrome?
Pulmonary hypertension with reversal of shunt is called Eisenmenger’s syndrome.
* Long Cases in Clinical Medicine, ABM Abdullah Page: 233
What is Eisenmenger’s complex?
Eisenmenger’s syndrome, when due to VSD is called Eisenmenger’s complex.
* Short and Long Cases in Clinical Medicine, HN Sarker Page: 62
What are the causes of Eisenmenger’s syndrome?
The causes include
- Atrial septal defect
- Ventricular septal defect
- Patent ductus arteriosus.
Note:
In VSD, this occurs in early life, in PDA a little later than VSD, in ASD this occurs in adult life.
* Short and Long Cases in Clinical Medicine, HN Sarker Page: 62; Long Cases in Clinical Medicine, ABM Abdullah Page: 233
What happens to the murmur of VSD as Eisenmenger’s complex develops?
With the development of pulmonary hypertension, left- to-right shunting decreases, murmur becomes shorter and soft and when pressure equalizes, then murmur disappears.
* Short and Long Cases in Clinical Medicine, HN Sarker Page: 62
What are the clinical features of Eisenmenger’s syndrome?
As follows:
- Dyspnea
- Fatigue
- Syncope
- Angina
- Hemoptysis
- Features of CCF.
On examination:
- Central cyanosis (not corrected by giving 100% oxygen. Differential cyanosis (cyanosis in toes, not in the hand) occurs in PDA.
- Clubbing (differential clubbing—clubbing in toes, not in the hand, occurs in PDA).
- Pulse—low volume.
- Prominent “a” wave in JVP.
- Other signs of pulmonary hypertension – palpable P2, left parasternal lift, epigastric pulsation due to RVH. Ejection click and ejection systolic murmur may be present.
- TR may occur (in such case, prominent V wave in JVP, also there may be a pansystolic murmur in left lower parasternal area).
- Polycythemia
- Original murmur of VSD, ASD or PDA—decrease in intensity, even may disappear.
Note:
If any patient is having cyanosis with evidences of PH, the more likely diagnosis is Eisenmenger syndrome.
* Short Cases in Clinical Medicine, ABM Abdullah, 5th Edition Page: 97
Why is second heart sound is single and loud?
P2 is loud due to pulmonary hypertension and A2 and P2 occur simultaneously because of equalization of left and right ventricular pressure.
* Short and Long Cases in Clinical Medicine, HN Sarker Page: 62
How would you differentiate Eisenmenger’s syndrome from TOF clinically?
Clinical differences between Eisenmenger’s syndrome and Fallot’s tetralogy
| Clinical features | Eisenmenger’s syndrome | Eisenmenger’s syndrome |
| Age | Adult/middle age | Children/adolescent, rarely adult |
| Venous pressure | Prominent a waves (if in sinus rhythm), even
V wave |
Absent a waves |
| Apex beat | Displaced | Undisplaced |
| Thrill in pulmonary area | Systolic thrill is present | Systolic thrill is present |
| Murmur in pulmonary area
|
Murmur is absent | Ejection systolic murmur is present |
| Characteristics of S2 | Loud P2. Fixed spitting with ASD; reversed splitting with PDA; and single loud S with VSD | Single A2 (with diminished or absent P2) |
* Short and Long Cases in Clinical Medicine, HN Sarker Page: 63
What investigations do you suggest in this case?
As follows:
- X-ray chest (enlargement of central pulmonary arteries with peripheral pruning of pulmonary vessels).
- ECG (RVH, RAH, right axis deviation).
- Echocardiography
* Long Cases in Clinical Medicine, ABM Abdullah Page: 233
Why do you think Eisenmenger’s syndrome?
Presence of central cyanosis, clubbing of fingers and toes with evidence of pulmonary hypertension suggest Eisenmenger’s syndrome.
* Short and Long Cases in Clinical Medicine, HN Sarker Page: 62
Why do you think VSD is the underlying cause?
Single loud second heart sound suggests VSD as the underlying cause.
* Short and Long Cases in Clinical Medicine, HN Sarker Page: 62
Why is Eisenmenger’s syndrome not due to ASD in this patient?
In ASD, S2 is fixed and widely split (A2–P2) due to equalization of right and left atrial pressures, thereby losing respiratory effect on S2.
* Short and Long Cases in Clinical Medicine, HN Sarker Page: 62
Why is Eisenmenger’s syndrome not due to PAD in this patient?
In PDA, there is reverse splitting of second heart sound (P2-A2) as a result of delayed emptying of left ventricle due to volume overload and presence of differential cyanosis and clubbing, i.e. cyanosis and clubbing of toes but not of the fingers.
* Short and Long Cases in Clinical Medicine, HN Sarker Page: 62
What are your differential diagnoses?
As follows:
- Congestive cardiac failure
- Chronic cor pulmonale.
* Long Cases in Clinical Medicine, ABM Abdullah Page: 232
Why not CCF?
In CCF, the triad of engorged and pulsatile neck vein, enlarged tender liver and dependent edema should be present. It is usually secondary to other causes like mitral stenosis or left sided heart failure, which are absent in this case.
* Long Cases in Clinical Medicine, ABM Abdullah Page: 233
Why not this is a case of chronic cor-pulmonale?
Cor pulmonale is defined as enlargement of right ventricle with or without failure which may be due to causes in the lung parenchyma, pulmonary vessels or chest wall (like kyphosis, scoliosis, etc.). All of these are absent in this case.
* Long Cases in Clinical Medicine, ABM Abdullah Page: 233
How to treat Eisenmenger’s syndrome?
The management includes
Conservative management
- Avoid dehydration, contraception and pregnancy
- Prophylaxis for infective endocarditis
- Oxygen therapy
- Diuretic therapy for heart failure
- Anticoagulation
- Venesection for polycythemia and hyperviscosity
- Vasodilator therapy for pulmonary hypertension.
Surgical management
- Surgical repair of primary cardiac defect if the pulmonary hypertension is reversible
- Heart-lung transplantation if pulmonary hypertension is irreversible.
Note:
Surgery of shunt is contraindicated in Eisenmenger’s syndrome, as it aggravates right sided heart failure.
* Short and Long Cases in Clinical Medicine, HN Sarker Page: 63; Long Cases in Clinical Medicine, ABM Abdullah Page: 234
What are the complications of Eisenmenger’s syndrome?
The complications include
- Hemoptysis
- Right ventricular failure
- Paradoxical embolism
- Infective endocarditis
- Polycythemia
- Thrombosis
* Short and Long Cases in Clinical Medicine, HN Sarker Page: 62
What are the causes of death in Eisenmenger’s syndrome?
As follows:
- Right heart failure
- Infective endocarditis
- Pulmonary infarction
- Cerebral thrombosis or abscess
- Arrhythmias
* Long Cases in Clinical Medicine, ABM Abdullah Page: 234
Eisenmenger’s syndrome
- If a patient has cyanosis with evidences of pulmonary hypertension, the more likely diagnosis is Eisenmenger’s syndrome.
- If a patient with VSD develops Eisenmenger’s syndrome, there is appearance of cyanosis, clubbing and evidences of pulmonary hypertension. However, pansystolic murmur of VSD may disappear, because of equalization of pressure between right and left ventricles.
- Patient with Eisenmenger’s syndrome is at particular risk from abrupt changes in afterload that exacerbate right to left shunting (e.g. during anesthesia and also in pregnancy).
* Long Cases in Clinical Medicine, ABM Abdullah Page: 234
