Capillary leak syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: M. Hassan, M.B.B.S

Natural History

The episode usually consists of two phases

The Capillary Leak Phase (1-4 days)

The initial phase is the capillary leak phase, lasting from 1 to 4 days. Clinical features are abdominal pain, nausea, generalized edema and hypotension that may result in cardiopulmonary collapse. Acute renal failure is due to acute tubular necrosis consequent to hypovolemia and rhabdomyolysis.

Recruitment of the Interstitial Fluid

The second phase results in the recruitment of the initially extravasated fluid. Intravascular overload with polyuria and pulmonary edema often occur. Edema may be more severe due to massive fluid supply in the initial phase. It's necessary to monitor the patient in order to switch to depletion treatment with diuretics or hemofiltration.

Primary MR

What is the severity of MR?

Severe MR ❑ Severe MVP with loss of coaptation ❑ RHD with loss of central coaptation ❑ Left ventricular dilation ❑ Regurgitation fraction ≥ 50% ❑ Regurgitation volume ≥ 60ml ❑ Effective regurgitation orifice ≥ 0.4cm² ❑ Vena contracta ≥ 0.7cm

Progressive MR (Stage B) ❑ Severe MVP with normal coaptation ❑ RHD with normal coaptation ❑ No Left ventricular dilation ❑ Regurgitation fraction < 50% ❑ Regurgitation volume < 60ml ❑ Effective regurgitation orifice < 0.4cm² ❑ Vena contracta < 0.7cm

Is the patient symptomatic?

Yes (Stage D)

No (Stage C)

Is the LVEF>30%?

LVEF 30-60% OR LVESD≥40 mm (Stage C2)

LVEF>60% AND LVESD<40 mm (Stage C1)

New onset atrial fibrillation OR PASP>50 mmHg (Stage C1)

Is the likelihood of success for the valve repair >95% and the expected mortality <1%?

No

Yes

Yes

No

Mitral valve surgery (Class IIb)

Mitral valve surgery (Class I)

Mitral valve repair (Class IIa)

Periodic monitoring

Periodic monitoring

Indications for Surgery in Chronic Secondary Mitral Regurgitation

Shown below is an algorithm depicting the indications for mitral valve surgery or period monitoring among patients with chronic secondary MR according to the 2014 AHA/ACC guideline for the management of patients with valvular heart disease. Note that when mitral valve surgery is indicated, mitral valve repair is preferred over mitral valve replacement whenever feasible.

Abbreviations: MR: mitral regurgitation

Secondary MR and patient is receiving medical therapy

What is the severity of MR?

Symptomatic (NYHA class III-IV) severe MR (Stage D)

Asymptomatic severe MR (Stage C)

Progressive MR (Stage B)

Mitral valve surgery (Class IIb)

Periodic monitoring

Periodic monitoring

Complications

Most common complications of capillary leak syndrome include the following:

Complications of Capillary Leak Syndrome
Compartment syndrome Pulmonary edema End-organ damage leading to multiorgan failure Arrhythmias Pericardial effusion Myocardial edema Pancreatitis Acute deep venous thrombosis Acute renal failure Rhabdomyolysis Pleural effusion Acute ischemic stroke

Prognosis

Mortality is reported in 21% of the 57 cases described. However, better management of this condition has recently led to lower mortality.

In the Mayo Clinic’s experience, the median survival of 25 patients that were followed over 30 years (counting only SCLS-related deaths) was approximately 15 years, and their 5-year survival rate was 76%. In European experience, the 5-year post-diagnosis survival rate was 85% in 23 patients who had received prophylactic treatment and 20% in 5 patients who had not. However, better identification and management of this condition appears to be resulting in lower mortality and improving survival and quality-of-life results as of late.

References

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