https://doi.org/10.1007/s00392-025-02625-4
1Universitätsklinikum Magdeburg A.ö.R. Klinik für Herz- und Thoraxchirurgie Magdeburg, Deutschland; 2Universitätsklinikum Magdeburg A.ö.R. Klinik für Kardiologie, Angiologie und Pneumologie Magdeburg, Deutschland; 3Universitätsklinikum Magdeburg A.Ö.R Universitätsklinik für Pneumologie Magdeburg, Deutschland
Aim:
Carcinoid heart disease (CHD) also known as Hedinger syndrome is a rare but challenging cardiac manifestation on neuroendocrine tumor which requires a complex therapeutical approach. We here aim to present our experience with a patient who underwent double valve replacement in our clinic.
Case Presentation:
Carcinoid heart disease (CHD) also known as Hedinger syndrome is a rare but challenging cardiac manifestation on neuroendocrine tumor which requires a complex therapeutical approach. We here aim to present our experience with a patient who underwent double valve replacement in our clinic.
Case Presentation:
A 62-year-old patient with shortness of breath and significant oedema of the lower limbs was admitted to the pulmonology department for further diagnosis. Upon admission severe hypoxemia was confirmed in the capillary blood gas analysis (pH 7.49, pO2 44 mmHg, pCO2 28 mmHg, sO2 81%). The patient had a history of neuroendocrine tumor of the ileum, where he underwent various treatment modalities including partial resection of the small intestine, peptide receptor radionuclide therapy (PRRT) as well as Sandostatin therapy. The Patient was also suffering from radiation induced pulmonary fibrosis.
After ruling out other lung pathologies and new metastasis with computed tomography and bronchoscopy, a transoesophageal echocardiography was performed, revealing typical characteristics of Hedinger syndrome, including a dilated right heart with significant tricuspid valve regurgitation and severe pulmonary valve regurgitation. A prominent patent foramen ovale (PFO) was also observed and confirmed with a contrast-enhanced ultrasound, which demonstrated a massive right-to-left shunt.
After discussing the case in our interdisciplinary team decision was made on a surgical correction of the valve pathologies with closure of the PFO. The patient underwent tricuspid and pulmonary valve replacement with bioprosthesis as well as a direct closure of the PFO. The Surgery was performed with a median sternotomy approach with standard aortic and bicaval venous cannulation. Valve repair was not attempted due to significant thickening of the valve leaflets, and bioprosthetic valves were chosen to avoid the need for long-term anticoagulation therapy. The immediate postoperative course was uneventful, aside from the requirement of a pacemaker due to an AV block. The Patient was transferred to normal ward on the 6th postoperative day (POD) and discharged home on the 10th POD. A follow-up after a six-month interval showed a drastic improvement in symptoms, with good oxygenation in blood gas analysis (pH 7.45, pO₂ 88 mmHg, pCO₂ 33 mmHg, and sO₂ 96%) and significantly increased exercise tolerance.
Conclusion:
Carcinoid heart disease represents a complex cardiac condition with a known poor prognosis. The underlying pathophysiology is multifactorial and intricate, with evidence suggesting that prolonged exposure of the endocardium to peptide hormones (such as serotonin) leads to fibrotic thickening and disruption of the valvular apparatus. This case highlights the critical role of interdisciplinary collaboration in achieving optimal patient outcomes. The coordinated efforts of the cardiology, pulmonology, and surgical teams enabled a rapid, patient tailored treatment strategy that significantly improved the patient’s symptoms and overall quality of life.