Vol 80, No 7-8 (2022)
Clinical vignette
Published online: 2022-06-23

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Staged treatment of carcinoid syndrome complicated with severe tricuspid regurgitation

Marcin Waligóra12, Bogdan Suder3, Wojciech Magoń12, Jakub Stępniewski12, Kamil Jonas12, Piotr Podolec4, Grzegorz Kopeć1
Pubmed: 35735072
Kardiol Pol 2022;80(7-8):867-868.

Abstract

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„ Clinical vignette

Staged treatment of carcinoid syndrome complicated with severe tricuspid regurgitation

Marcin Waligóra12Bogdan Suder3Wojciech Magoń12Jakub Stępniewski12Kamil Jonas12Piotr Podolec4Grzegorz Kopeć1
1Pulmonary Circulation Center, Department of Cardiac and Vascular Diseases, Faculty of Medicine, Jagiellonian University Medical College, John Paul II Hospital in Krakow, Kraków, Poland
2Center for Innovative Medical Education, Department of Medical Education, Faculty of Medicine, Jagiellonian University Medical College, Kraków, Poland
3Department of Cardiovascular Surgery and Transplantation, John Paul II Hospital, Kraków, Poland
4Department of Cardiac and Vascular Diseases, Faculty of Medicine, Jagiellonian University Medical College, John Paul II Hospital in Krakow, Kraków, Poland

Correspondence to:

Grzegorz Kopeć, MD, PhD,

Pulmonary Circulation Center,

Department of Cardiac and Vascular Diseases,

Institute of Cardiology,

Jagiellonian University Medical College,

Prądnicka 80, 31–202 Kraków, Poland,

phone: +48 12 614 33 99,

e-mail: grzegorzkrakow1@gmail.com

Copyright by the Author(s), 2022

DOI: 10.33963/KP.a2022.0157

Received: May 5, 2022

Accepted: May 27, 2022

Early publication date: June 23, 2022

We present a case of a 49-year-old male with symptomatic carcinoid heart disease (CHD), a rare complication of gastrointestinal carcinoid syndrome, which occurs in 12 per 100 000 adults per year. The 5-year survival rate depends on staging, its origin, resectability, and the presence of CHD. CHD is characterized by structural damage to the right heart, which is an effect of biogenic amines secretion by the tumor. The presence of CHD is associated with poor survival of 11 months for patients presenting in New York Heart Association (NYHA) functional class IV, and the key prognostic factor is the function of the tricuspid valve [1].

Our patient was diagnosed with a primary tumor in a small intestine with metastases to the liver, bone, and peritoneum and was treated in cooperation with a reference endocrinology center with a somatostatin analog. The serum level of urinary 5-hydroxyindoleacetic acid, the main metabolite of serotonin, was 329.3 mg/24 hours (N: 29 mg/24 hours). He presented with facial flushing, diarrhea, and persistent facial plethora on the cheeks, forehead, and chin (Supplementary material, Figure S1). He had severe functional impairment: NYHA class III, 6-minute walk test (6MWT) of 180 m, and maximal oxygen consumption of 10.4 ml/kg/min as assessed by ergospirometry. Serum concentration of N-terminal pro-B-type natriuretic peptide was elevated (1326 pg/ml, N: <125 pg/ml).

Echocardiography showed dilation of the right ventricle and right atrium, preserved right ventricular function, moderate stenosis and regurgitation of the pulmonary valve, severe tricuspid regurgitation [2] with thickened, retracted immobile leaflets with a coaptation gap of 13 mm (Figure 1AC). Right heart catheterization (RHC) showed a reduced cardiac index (1.75 l/min/m2), normal mean pulmonary artery pressure (16 mm Hg), pulmonary vascular resistance (2.2 Wood units), and pulmonary artery wedge pressure (8 mm Hg).

5286.png
Figure 1. A. Normal right ventricle contractility presented as tricuspid annulus plane systolic excursion (TAPSE) of 27 mm. B. Coaptation gap of tricuspid valve leaflets. Enlargement of right atrium (area of 37 cm2) and right ventricle (basal dimension in apical 4-chamber view of 50 mm and right ventricular outflow tract diameter of 47 mm). C. Colour flow image of severe tricuspid regurgitation of native valve (the red arrow). D. Trace tricuspid regurgitation within first day after successful tricuspid valve replacement (the green arrow). E. Improvement in chamber and heart geometry after surgical tricuspid valve replacement (right atrium area of 19 cm2, right ventricular outflow tract diameter of 40 mm and basal dimension in apical 4-chamber view of 44 mm). F. Moderate bioprosthesis regurgitation at 8-month follow-up (the orange arrow)
Abbreviations: RA, right atrium; RV, right ventricle

Due to symptomatic tricuspid regurgitation and significant exercise limitation, the perioperative risk of abdominal surgery was considered high [3, 4]. Accordingly, the Heart Team proposed that tricuspid valve replacement should precede abdominal surgery.

The patient was operated on with the use of extracorporeal circulation through the right atrial access. In the operation field, we could see the remains of the tricuspid leaflets adhered to the tricuspid annulus. A Perimount 29 M bioprosthesis was implanted successfully with a trace tricuspid regurgitation (Figure 1D). The following hospital stay was uneventful.

Four months after cardiac surgery the patient underwent successful partial resection of the ileum occupied by the primary tumor. Two months afterward, he was reassessed and presented a significant improvement in exercise capacity (NYHA, class II; 6MWD, 375 m). Echocardiography showed a normal gradient of bioprosthesis and a noticeable improvement in heart geometry (Figure 1E). RHC showed an improved cardiac index of 2.31 l/min/m2. We observed a relapse of the tricuspid regurgitation, which was moderate and stable at the 8-month follow-up (Figure 1F).

In our patient, cardiac surgery facilitated a clinical improvement and let the patient be safely operated on for an abdominal tumor. However, at follow-up, we observed deterioration of the tricuspid valve, which could result from the delay between cardiac and abdominal surgery and exposure of the prosthetic valve to high levels of serotonin metabolites before tumor resection [5]. Our results show the need for immediate tumor resection after valvular replacement and strict observation of the tricuspid prosthesis structure and function at follow-up.

Supplementary material

Supplementary material is available at https://journals.viamedica.pl/kardiologia_polska.

Article information

Conflict of interest: None declared.

Funding: None.

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REFERENCES

  1. Westberg G, Wängberg B, Ahlman H, et al. Prediction of prognosis by echocardiography in patients with midgut carcinoid syndrome. Br J Surg. 2001; 88(6): 865872, doi: 10.1046/j.0007-1323.2001.01798.x, indexed in Pubmed: 11412260.
  2. Rdzanek A, Szymański P, Gackowski A, et al. Percutaneous tricusid edge-to-edge reair Patient selection, imaging considerations, and the rocedural technique. Exert oinion of the Working Grou on Echocardiograhy and Association of Cardiovascular Interventions of the Polish Cardiac Society. Kardiol Pol. 2021; 79(10): 11781191, doi: 10.33963/KP.a2021.0125, indexed in Pubmed: 34611879.
  3. Davar J, Connolly HM, Caplin ME, et al. Diagnosing and managing carcinoid heart disease in patients with neuroendocrine tumors: an expert statement. J Am Coll Cardiol. 2017; 69(10): 12881304, doi: 10.1016/j.jacc.2016.12.030, indexed in Pubmed: 28279296.
  4. Parikh P, Banerjee K, Ali A, et al. Impact of tricuspid regurgitation on postoperative outcomes after non-cardiac surgeries. Open Heart. 2020; 7(1): e001183, doi: 10.1136/openhrt-2019-001183, indexed in Pubmed: 32399250.
  5. Castillo JG, Filsoufi F, Rahmanian PB, et al. Early bioprosthetic valve deterioration after carcinoid plaque deposition. Ann Thorac Surg. 2009; 87(1): 321, doi: 10.1016/j.athoracsur.2008.04.061, indexed in Pubmed: 19101329.



Polish Heart Journal (Kardiologia Polska)