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Pulmonary Valve Replacement After Repaired Tetralogy of Fallot

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Pulmonary Valve Replacement After Repaired Tetralogy of Fallot General Thoracic and Cardiovascular Surgery https://doi.org/10.1007/s11748-018-0931-0 REVIEW ARTICLE Pulmonary valve replacement after repaired Tetralogy of Fallot Hideki Tatewaki1 · Akira Shiose1 Received: 17 February 2018 / Accepted: 28 April 2018 © The Japanese Association for Thoracic Surgery 2018 Abstract In this review article, we describe pulmonary valve replacement (PVR) late after repaired Tetralogy of Fallot (TOF). Since the introduction of surgical intervention for patients with TOF in 1945, surgical management of TOF has dramatically improved early survival with mortality rates, less than 2–3%. However, the majority of these patients continue to experience residual right ventricular outflow tract pathology, most commonly pulmonary valve regurgitation (PR). The patients are generally asymptomatic during childhood and adolescence and, however, are at risk for severe PR later which can result in exercise intolerance, heart failure, arrhythmias, and sudden death. While it has been shown that PVR improves symptoms and functional status in these patients, the optimal timing and indications for PVR after repaired TOF are still debated. This article reviews the current state of management for the patient with PR after repaired TOF. Keywords Tetralogy of Fallot · Pulmonary regurgitation · Pulmonary valve replacement · Adult congenital heart disease · Sudden death Introduction integral tool in evaluation of RV volumes, severity of PR, and biventricular function in following these patients later Tetralogy of Fallot (TOF) is the most common form of in life. It has been shown that Pulmonary valve replace- cyanotic congenital heart disease. The first Blalock–Taus- ment (PVR) alleviates symptoms, normalizes RV volumes, sig shunt operation in 1945 improved life expectancy from and improves RV function. It is unclear whether perform- a few months to years [1]. Lillehei et al. followed with the ing PVR decreases the risk of ventricular tachycardia (VT) first successful intracardiac repair in 1954 [2]. The current or sudden death. There is strong evidence that elimination early survival of TOF is excellent, at approximately 90–98% or significant reduction of PR after PVR is associated with through the first two decades of life [3, 4]. While early mor- symptomatic improvement, decrease in RV end-diastolic tality has improved, studies of adult survivors with repaired volume (RVEDV) and RV endo-systolic volume (RVESV), TOF showed that late complications included exercise intol- and no significant change in RV ejection fraction using erance, right heart failure, arrhythmias and sudden death [5, CMR imaging [5, 10–12]. There is no consensus, however, 6]. Many of these complications are related to the hemo- regarding the indication and the timing for PVR. This article dynamic effects of pulmonary regurgitation (PR) and asso- reviews the current state of management for the patient with ciated chronic right ventricular (RV) volume overload [5, PR after repaired TOF. 7, 8]. Patients with PR can remain asymptomatic for many years with a gradual deterioration of exercise performance inversely related to PR severity [9]. Because echocardiogra- Pulmonary regurgitation phy is often inadequate for evaluating early RV dysfunction, cardiac magnetic resonance (CMR) imaging has become an After intracardiac repair of TOF, the patient nearly always had residual right ventricular outflow tract (RVOT) pathol- ogy, most commonly PR. For many years, PR after repaired * Hideki Tatewaki TOF was considered benign [13]. Young patients appeared [email protected] to tolerate PR well and were subsequently lost to follow-up. 1 Department of Cardiovascular Surgery, Kyushu University It was later found that as these patients reached adulthood, Hospital, 3-1-1 Maidashi, Higashi-ku, Fukuoka 8128582, the hemodynamic burden of chronic PR presented as late Japan Vol.:(0123456789)1 3 General Thoracic and Cardiovascular Surgery morbidity including exercise intolerance, right heart failure, RV makes determining volume and function difficult using and arrhythmias. The most common cause of mortality was echocardiographic assessments. Doppler echocardiographic sudden death. These adverse long-term outcomes in patients assessment of PR is also dependent on the observer’s expe- with TOF are demonstrated by a significant decrease in their rience, and is limited by post-operative structural changes. survival curve in the second decade following repair [3]. CMR imaging has become the reference standard for assess- RV systolic function in chronic PR is initially preserved. ment of biventricular size and function and offers a reliable This stage may last for years and many patients remain rela- method for the longitudinal assessment of PR severity and tively free of symptoms. As PR progresses, however, several RV dilation in patients with repaired TOF [10, 20, 21]. Fur- studies have shown a close relationship between the severity thermore, CMR imaging is non-invasive and does not use of PR and RV end-diastolic volume [14, 15]. Severe PR is ionizing radiation. Echocardiography and CMR imaging also known to impair biventricular function. Davlouros et al. can be used in concert as a combined non-invasive imaging demonstrated that left ventricular (LV) systolic dysfunction study in patients with repaired TOF. Yamasaki et al. dem- correlated with RV dysfunction in adults with repaired TOF, onstrated that cardiac computed tomography (CCT) can suggesting an unfavorable ventricular–ventricular interaction also assess RV function and pulmonary regurgitant frac- [16]. This study also showed three independent predictors of tion (PRF), compared with CMR imaging in patients with LVEF: length of time that patient remained palliated, aortic repaired TOF [22]. CCT is more useful than CMR for the regurgitation fraction, and RVEF; the first two correlated patients with an implanted pacemaker/defibrillator, patients inversely with LVEF. The mechanism that links RV dysfunc- with claustrophobia or any clinical condition that prohibits tion to LV dysfunction, however, is incompletely understood a long CMR examination. in repaired TOF. Indication and timing for PVR Arrhythmias PVR can be accomplished with a low early mortality in Arrhythmias are also common sequelae of repaired TOF. many centers, but should be performed before there is irre- Atrial arrhythmias may be present in approximately one- versible RV dysfunction and the increased propensity for third of patients and are a major source of morbidity. VT and sudden death [23, 24]. The optimal management of Although the prevalence of sustained ventricular tachycardia patients who may have clinical or physiological indications (VT) is low, it is believed to be responsible for an increased for PVR has been widely researched and debated. Identifica- incidence of sudden death in patients with TOF. Gatzoulis tion of risk factors for adverse clinical events is important for et al. in a multicenter study of 793 patients with repaired determining the optimal timing of PVR. There is agreement TOF at a mean of 8.2 ± 8 years, demonstrated that an older that PVR is indicated in symptomatic patients with signifi- age at TOF repair and QRS duration > 180 ms were inde- cant PR, heart failure, or new arrhythmias. In the absence pendent predictors of sudden death [5]. Khairy et al. in a of these findings, physicians should consider a broad range multi-institutional study in 556 patients with repaired TOF of clinical data and longitudinal imaging modalities when reported a prevalence of atrial tachyarrhythmias (20.1%) and considering PVR in the individual patient. ventricular arrhythmias (14.6%) that markedly increased Several CMR imaging based studies have found that RV after 45 years of age. These arrhythmias were associated volumes return to normal after PVR if the pre-operative with the number of cardiac surgeries, QRS duration, and left RVEDVI is < 150–170 ml/m2 or the RVESVI is < 82–90 ml ventricular diastolic dysfunction [17]. Targeted intraopera- l/m2 [5, 10, 12]. Biventricular dysfunction and RV hyper- tive cryoablation procedures during PVR appear to decrease trophy are associated with poor outcome in patients with the incidence of arrhythmias, at least in the short term [18]. repaired TOF [25]. In patients with risk factors for VT and sudden death, pre- Frigiola et al. advocated that a relatively aggressive PVR operative electrophysiological study and implantation of a indicator (RVEDVI < 150 ml/m2) leads to normalization of cardioverter–defibrillator should be considered [19]. RV volumes, improvement in biventricular function, and increased exercise capacity [26]. Frigiola also reported that patients with repaired TOF who were > 35 years of age with- Evaluation of pulmonary regurgitation out PVR and with normal exercise capacity showed mild and ventricular function residual RVOT obstruction and pulmonary annulus diam- eters < 0.5Z [27]. For many years, echocardiography has been used as the Lee et al. suggest that pre-operative RVEDVI of 168 ml/ primary non-invasive imaging modality in the follow-up of m2 and pre-operative RVESVI < 80 ml/ m2 are predictors patients with repaired TOF. The complex geometry of the for optimal outcome [10]. RV volume normalization is also 1 3 General Thoracic and Cardiovascular Surgery associated with a pre-operative QRS duration < 140 ms, Other complications whereas a QRS duration > 160 ms and an RV ejection frac- tion < 45% are associated with persistent RV dilation and Coexisting problems such as tricuspid
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