Tricuspid valvectomy is a rare surgical intervention, and knowledge regarding long-term outcome in children is lacking. We report a favourable outcome 11 years after tricuspid valvectomy in early infancy without subsequent surgery or other cardiac interventions. Specific criteria for timing of re-intervention are lacking. Application of adult tricuspid and pulmonary regurgitation recommendations is helpful but has limitations

Bogers, Ad JJC

Helbing, Willem A

Vanhie, Ellen

English

Ghent University Academic Bibliography

Cardiology in the Youngwww.cambridge.org/ctyBrief ReportCite this article: Vanhie ESI, Bogers, AJJC,and Helbing WA. Long-term outcome aftertricuspid valvectomy in a neonate withCandida endocarditis: a case report.Cardiology in the Young 29: 528–530.doi: 10.1017/S1047951119000027Received: 2 November 2018Revised: 5 December 2018Accepted: 21 December 2018Key words:Tricuspid valve; valvectomy; endocarditis;childrenAuthor for correspondence:Willem A. Helbing, MD, PhD, Division ofPaediatric Cardiology, Department ofPaediatrics, Erasmus University MedicalCentre - Sophia Children’s Hospital, DoctorMolewaterplein 40, 3015 GD Rotterdam,The Netherlands. Tel: +31107036264;Fax: +31107036801;E-mail: w.a.helbing@erasmusmc.nl© Cambridge University Press 2019. This is anOpen Access article, distributed under theterms of the Creative Commons Attributionlicence (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted re-use,distribution, and reproduction in any medium,provided the original work is properly cited.Long-term outcome after tricuspid valvectomyin a neonate with Candida endocarditis: a casereportEllen S. I. Vanhie1,2, Ad J. J. C. Bogers,3 and Willem A. Helbing21Department of Paediatrics, University Hospital of Ghent, Ghent, Belgium; 2Division of Paediatric Cardiologyand Radiology, Department of Paediatrics, Erasmus University Medical Centre - Sophia Children’s Hospital,Rotterdam, The Netherlands and 3Department of Cardiothoracic Surgery, Erasmus University Medical Centre,Rotterdam, The NetherlandsAbstractTricuspid valvectomy is a rare surgical intervention, and knowledge regarding long-term out-come in children is lacking.We report a favourable outcome 11 years after tricuspid valvectomyin early infancy without subsequent surgery or other cardiac interventions. Specific criteria fortiming of re-intervention are lacking. Application of adult tricuspid and pulmonary regurgita-tion recommendations is helpful but has limitations.Isolated right-sided infective endocarditis is uncommon, especially in neonates.Candida speciesis the underlying micro-organism in 10% of infants with infective endocarditis. In neonates, thisresults in a right-sided endocarditis in 90% of the Candida endocarditis cases.1First choice treatment is anti-microbial medication. Surgery should be considered when largetricuspid valve vegetation is present, when antibiotic medical treatment fails, when the infectionis caused by micro-organisms difficult to eradicate, or when refractive right heart failure ispresent.2 The preferred surgical treatment is valve repair. Because of the long-term risk of devel-oping severe right heart failure, tricuspid valvectomy is considered only in exceptional cases.3Data on prognosis of living without tricuspid valve are scarce.3,4 Aim of this report is to sharea history of over 10-year survival after tricuspid valvectomy in infancy.Case reportWe describe the case of a female infant born at 29 weeks and 3 days of gestational age with birthweight of 1310 kg. Details of her medical history in the first months of life have been reportedpreviously.5At the age of 5 months, she was diagnosed with a thrombus obstructing the tricuspid orifice.Cardiac surgery was performed within 24 hours after admission. The tricuspid valve could notbe repaired and valvectomy was performed. Blood cultures and thrombus examination showeda Candida albicans, so fluconazole was initiated. The post-operative course was prolonged,requiring maximal support with inotropic medication, diuretics, nitric oxide, and sildenafil.Eventually she was discharged 5 weeks post-operatively using diuretics, sildenafil 8 mg/kg/day, and fluconazole. Ultrasonography at discharge showed a dilated right heart, with normalcardiac output and moderate reversed flow in the hepatic veins.5The following years the girl did surprisingly well. Mild pulmonic regurgitation was noted.Based on the measurements of pulmonic regurgitation flow velocity and the shape of the rightventricle, there were no signs of increased pulmonary artery pressure. Because of the combinedclinical and echo findings, we did not perform invasive measurements. Sildenafil dosage wasgradually reduced and sildenafil was stopped 1 year after surgery. With the diuretics as onlymedication, she had a normal growth pattern and exercise capacity during the subsequent years.There was no progressive dilatation of the right atrium or ventricle. Until the age of 11 years old,she stayed clinically stable without cardiac arrhythmias or signs of right heart failure.Since the age of 11 years, exercise intolerance is present. Cardiopulmonary exercise testshowed exercise intolerance with a peak workload of 100 W (predicted 140 W, 71%) and adecrease of peak oxygen uptake from 37 to 28 ml/kg/minute over the last 3 years (Table 1).There were no signs of ventricular arrhythmias (as confirmed on 24-hour Holter monitoring).Cardiac MRI showed non-progressive dilatation of the right ventricle and a normal ventricularejection fraction (Fig 1). Because of sub-optimal exercise performance and dilatation of the rightheart (Table 1), the question for surgical intervention arises. Based on criteria including thoseused in more common situations of right ventricular dilatation, such as pulmonary regurgita-tion, for now a conservative strategy is implemented, guided by the clinical state of the patient.https://doi.org/10.1017/S1047951119000027Downloaded from https://www.cambridge.org/core. Universiteit Gent, on 31 Mar 2020 at 10:03:35, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms.DiscussionInformation on long-term outcome after tricuspid valvectomy isscarce. Isolated tricuspid regurgitation is commonly well toleratedin adults.4 However, chronic severe tricuspid regurgitation maylead to volume overload of the right ventricle, which may causeventricular dilatation and loss of cardiac function. Symptomsmay appear only when the disease is already advanced.4 Chronicmedical treatment for severe isolated tricuspid regurgitation inadults consists of diuretics, and is used when clinical symptomsof right heart failure are present. There is no evidence that theuse of diuretics improves the survival rate in these patients.4,6Furthermore, diuretics might reduce clinical symptoms and besuccessful in reducing heart failure symptoms, which might resultin delay of surgical intervention and poorer outcome.7In our case, pulmonary hypertension, following tricuspidvalvectomy, initially persisted despite maximum support withdiuretics, inotropics, high oxygen ventilation, and inhaled NO.Eventually, weaning from respiratory support was successful afterintroduction of sildenafil 8 mg/kg/day. In the following year, thegirl was treated with diuretics and sildenafil to provide optimal pre-load and afterload of the right ventricle. There were no side effectsreported.Sildenafil is well studied and approved for pulmonary hyperten-sion in adults. Currently, large trials in term and pre-term neonatesare lacking.8 A Cochrane 2017 systematic review evaluated the effi-cacy of sildenafil monotherapy in lowering mortality in neonatescompared to placebo. Sildenafil as adjuvant therapy with other pul-monary vasodilating products did not change mortality rates.9Tricuspid valvectomy has never been the first choice treatmentoption for endocarditis.3 In our case, clinical state and heart func-tion remained normal for 10 years after tricuspid valvectomy. Thisfavourable outcome can be explained by the positive response onmedical treatment and absence of complications such as pulmo-nary embolism in the acute phase. Furthermore, chronic medicaltreatment with diuretics and new drugs such as sildenafil may haveimproved the outcome.8,9Table 1. Evolution of cardiopulmonary measurementsAge (years) 8 11MRILV end-diastolic volume (ml/m²) 61 74LV stroke volume (ml/m²/beat) 35 48LV ejection fraction (%) 58 65RV end-diastolic volume (ml/m²) 164 158RV stroke volume (ml/m²) 97 98RV ejection fraction (%) 59 62Tricuspid regurgitation (%) 64 51CPETRER peak >1.2 >1.1Maximal Power(W/kg) 2.9 2.3VO2 peak (ml/kg/minute) 37 28VO2 peak % of predicted value 88 76CPET= cardiopulmonary exercise testing; LV= left ventricle; RER peak= respiratory exchange ratio at peak exercise;RV= right ventricle.Figure 1. Still frames of end-diastolic images of short axis orientation steady-state free-precession cine cardiac magnetic resonance imaging performed in 2014 (a) and 2017 (b).Left ventricle is on the right-hand side of panels (a) and (b).Cardiology in the Young 529https://doi.org/10.1017/S1047951119000027Downloaded from https://www.cambridge.org/core. Universiteit Gent, on 31 Mar 2020 at 10:03:35, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms.There is no guideline concerning timing of surgical re-interven-tion after tricuspid valvectomy.4 Our case shows a long-termsurvival with possibility to postpone surgery, in a young child.The AHA/ACC and ESC/EACTS guidelines for adults recom-mend surgical intervention for primary, isolated tricuspid regurgi-tation when patients are symptomatic or, if they are asymptomatic,when there is progressive right ventricular dilatation and/ordysfunction (class IIa recommendation, level of evidence C).Surgery needs to be performed as soon as the indication is set,to avoid irreversible right ventricular dysfunction.6,7Follow-up includes imaging with echocardiography and MRI.The 2017 ESC/EACTS guideline describes cut-off values of echo-cardiographic measurements defining severe valve regurgitation inadults.6,7 The lack of cut-off values for children is striking. Thisbears resemblance to the setting of chronic right ventricle volumeoverload following chronic pulmonary regurgitation, in which sizecriteria of the right ventricle as indication for intervention are sub-ject of continued debate.10 This hampers decision-making in a caselike this. In analogy to the situation in valve replacement in pulmo-nary regurgitation, we will opt for tricuspid valve replacement ifsymptoms, exercise test results, right ventricular size, and functionand/or ECG changes all point towards progressive decline of rightventricular function.ConclusionThis case illustrates the ability for survival in good clinical condi-tion for more than 10 years after tricuspid valvectomy performedin infancy. Growth, exercise capacity, and right ventricular size andfunction remained within acceptable limits. Consensus criteria fortiming of re-intervention are lacking.Financial Support. This research received no specific grant from any fundingagency, commercial or not-for-profit sectors.Conflicts of Interest. None.Ethical Standards. Written informed consent was obtained.References1. Anil Kumar V, Francis E, Sreehari S, Raj B. Tricuspid valve mycetoma inan infant successfully treated by excision and complex tricuspid valverepair followed by fluconazole therapy. Surg Infect (Larchmt) 2014; 15:134–140.2. Habib G, Lancellotti P, Iung B. 2015 ESC guidelines on the management ofinfective endocarditis: a big step forward for an old disease. Heart 2016; 102:992–994.3. YongMS, Coffey S, Prendergast BD, et al. Surgical management of tricuspidvalve endocarditis in the current era: a review. Int J Cardiol 2016; 202:44–48.4. Rodes-Cabau J, Taramasso M, O’Gara PT. Diagnosis and treatment of tri-cuspid valve disease: current and future perspectives. Lancet 2016; 388:2431–2442.5. du Plessis FA, Helbing WA, Bogers AJ. Excision of the tricuspid valve in ababy with Candida endocarditis. Cardiol Young 2007; 17: 545–547.6. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline forthe management of patients with valvular heart disease: a report of theAmerican College of Cardiology/American Heart Association Task Forceon Practice Guidelines. J Thoracic Cardiovas Surg 2014; 148: e1–e132.7. Baumgartner H, Falk V, Bax JJ, et al. 2017 ESC/EACTS guidelines for themanagement of valvular heart disease. Eur Heart J 2017; 38: 2739–2791.8. Perez KM, LaughonM. Sildenafil in term and premature infants: a system-atic review. Clin Ther 2015; 37: 2598–2607.9. Kelly LE, Ohlsson A, Shah PS. Sildenafil for pulmonary hypertension inneonates. Cochrane Database Syst Rev 2017; 8: CD005494.10. Tretter JT, Friedberg MK, Wald RM, McElhinney DB. Defining and refin-ing indications for transcatheter pulmonary valve replacement in patientswith repaired tetralogy of Fallot: contributions from anatomical and func-tional imaging. Int J Cardiol 2016; 221: 916–925.530 E. S. I. Vanhie et al.https://doi.org/10.1017/S1047951119000027Downloaded from https://www.cambridge.org/core. Universiteit Gent, on 31 Mar 2020 at 10:03:35, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms.

Long-term outcome after tricuspid valvectomy in a neonate with Candida endocarditis : a case report

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Long-term outcome after tricuspid valvectomy in a neonate with Candida endocarditis : a case report

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