Myocardial fibrosis (MF) and necrosis of the left heart has been reported as CF-related complication, especially in postmortem case series (Witt et al. 2000).
Discussion
We report a 9-year-old, pancreatic insufficient, malnourished CF-boy [F508del/CFTRdele2,3(21kb)], who presented with dyspnoea, cough and cardiac arrhythmia without pulmonary exacerbation. He had a normal lung function (FEV1%pred 86%) and no chronic Pseudomonas (Ps.) infection. Ventricular tachycardia and isolated left heart failure with dilatation of the left ventricle (ejection fraction 28%) were diagnosed. Myocarditis or inherited cardiomyopathy were made unlikely. He underwent a pulmonary banding, received an implantable cardioverter-defibrillator and was listed for heart transplantation. Under anticongestive therapy he remained stable until an uncomplicated orthotopic heart transplantation at the age of 12 years. Histological examination of explanted heart showed no inflammation, no malignoma – but obvious interstitial MF. Immunosuppression included tacrolimus, mycophenolate and initial prednisone. No acute or chronic rejection occurred over 3.5 years. Intermittent infection with Ps. aeruginosa was treated by eradication therapy. Under long-term antibiotic prophylaxis against Staphylococcus his lung function remained stable (last FEV1%pred 79%). His height increased (<3. to 10. percentile), but underweight remained (<3. percentile over time).
Conclusion
Isolated left heart failure is a rare, but life-threatening complication in CF. Isolated heart transplantation could be performed successfully without negative impact on lung disease due to extensive immunosuppression.
