Borowski A, Ghodsizad A, Litmathe J, Lawrenz W, Schmidt K G, Gams E
Clinic of Thoracic and Cardiovascular Surgery, University of Duesseldorf, Duesseldorf, Germany.
Pediatr Cardiol. 2004 Sep-Oct;25(5):466-71. doi: 10.1007/s00246-003-0579-z. Epub 2004 Mar 4.
After total repair of tetralogy of Fallot (TOF-R) with transannular patching (TAP), severe pulmonary regurgitation (PR) is reported to develop in up to 30% of patients at a follow-up of 20 years, and 10-15% or more need pulmonary valve replacement (PVR). In this study, time-related progression of PR and right ventricular (RV) dilatation, and functional recovery of the RV after PVR are analyzed, and the possible causes of PR and timing of PVR are discussed.
Eighteen patients, who late after TOF-R with TAP underwent PVR for severe PR, were chosen for the study. NYHA class, QRS duration, RV dilatation index (RVDI = RVEDD/LVEDD), and RV-distal pulmonary artery (PA) peak systolic gradient were reviewed and retrospectively analyzed.
TOF-R was performed at a mean age of 5.1 +/- 3.9 years (range: 0.6-12.8 years); the mean time interval from TOF-R to PR grade 3 onset was 11.8 +/- 7.0 years (range: 3.3-27.4 years), and from TOF-R to PVR was 18.5 +/- 7.8 years (range: 8.7-37.1 years). At PVR, 11 patients were in NYHA class II-III, all patients had severe PR (grade 3/3) and severe RV enlargement, 4 patients had ventricular arrhythmias, 7 patients significant distal pulmonary artery stenosis, and 2 patients small nonrelevant residual VSD. The mean preoperative RVDI (normal: 0.5) was 0.99 +/- 0.14 (range: 0.75-1.3), the mean QRS duration 170 +/- 24 ms (140-220 ms), and the mean RV-distal PA peak systolic pressure gradient 33.3 +/- 19.0 mmHg (range: 10-60 mmHg). Patients aged at TOF-R> 5 years had considerably longer redo-free intervals than their younger counterparts: mean 23.1 years (range 8.7-37.1 years) vs 14.8 years (range: 9.3-21.2 years), respectively. The redo-free intervals and the duration of severe PR correlated inversely with the RV-PA gradient. At a mean follow-up of 1.3 years (2 weeks-5 years), the mean RVDI decreased from 0.99 +/- 0.14 to 0.69 +/- 0.15, the mean validity class improved from 2.5 to 1.1. One patient died.
After TOF-R with TAP, the progression of PR has very individual dynamics, resulting in extremely varying redo-free intervals. Concomitant pulmonary stenosis seems to exaggerate progression of PR. PVR results in effective reduction of diastolic dimensions of severely dilated RV and in improvement of validity class. Referred PVR in no-risk cases seems to be justified.
据报道，在采用跨环补片（TAP）进行法洛四联症根治术（TOF-R）后，20年随访时高达30%的患者会出现严重的肺动脉反流（PR），10% - 15%或更多患者需要进行肺动脉瓣置换术（PVR）。在本研究中，分析了PR和右心室（RV）扩张的时间相关进展以及PVR后RV的功能恢复情况，并讨论了PR的可能原因和PVR的时机。
选择18例TOF-R伴TAP术后因严重PR接受PVR的患者进行研究。回顾并回顾性分析纽约心脏协会（NYHA）心功能分级、QRS时限、RV扩张指数（RVDI = RVEDD/LVEDD）以及RV-远端肺动脉（PA）收缩期峰值梯度。
TOF-R手术时的平均年龄为5.1±3.9岁（范围：0.6 - 12.8岁）；从TOF-R到PR 3级发作的平均时间间隔为11.8±7.0年（范围：3.3 - 27.4年），从TOF-R到PVR的时间为18.5±7.8年（范围：8.7 - 37.1年）。在PVR时，11例患者为NYHAⅡ - Ⅲ级，所有患者均有严重PR（3/3级）和严重RV扩大，4例患者有心室心律失常，7例患者有明显的远端肺动脉狭窄，2例患者有小的无关残余室间隔缺损（VSD）。术前平均RVDI（正常：0.5）为0.99±0.14（范围：0.75 - 1.3），平均QRS时限为170±24 ms（140 - 220 ms），平均RV-远端PA收缩期峰值压力梯度为33.3±19.0 mmHg（范围：10 - 60 mmHg）。TOF-R时年龄>5岁的患者无再次手术间隔时间比年轻患者长得多：分别为平均23.1年（范围8.7 - 37.1年）和14.8年（范围：9.3 - 21.2年）。无再次手术间隔时间和严重PR的持续时间与RV-PA梯度呈负相关。平均随访1.3年（2周 - 5年）时，平均RVDI从0.99±0.14降至0.69±0.15，平均心功能分级从2.5改善至1.1。1例患者死亡。
在采用TAP进行TOF-R后，PR的进展具有非常个体化的动态变化，导致无再次手术间隔时间差异极大。合并的肺动脉狭窄似乎会加剧PR的进展。PVR可有效减小严重扩张的RV的舒张期尺寸并改善心功能分级。在无风险病例中推荐PVR似乎是合理的。
