今日の臨床サポート

Fallot四徴症(小児科)

著者: 小野博 国立成育医療研究センター 循環器科

監修: 五十嵐隆 国立成育医療研究センター

著者校正/監修レビュー済:2019/07/09
参考ガイドライン:
  1. 日本循環器学会:成人先天性心疾患診療ガイドライン
  1. 日本循環器学会:先天性心疾患術後遠隔期の管理・侵襲的治療に関するガイドライン
  1. 日本循環器学会:先天性心疾患の診断、病態把握、治療選択のための検査法の選択ガイドライン
  1. ACC/AHA ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease
  1. ESCESC Guidelines for the management of grown-up congenital heart disease
患者向け説明資料

概要・推奨   

  1. ファロー四徴の心内修復術の至適時期は生後3~11カ月である(推奨度1)
  1. ファロー四徴の患者をみたら、22q11.2欠失症候群を念頭に置く。その後の発達異常や精神異常が出現するため、顔貌や低カルシウム血症などから22q11.2欠失症候群を疑ったら、染色体検査を施行すべきである
  1. spellはカテーテル検査時、上室頻拍時、朝、夏、感染を契機に起こりやすい。カテーテル検査時は塩酸ペチジンの前投薬が有用。発作時は鎮静、末梢血管収縮薬などの投与が必要(推奨度1)
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要
  1. 多くの場合は良好に修復されているため、妊娠・出産リスクは一般妊娠に近い[1](エビデンスランクOJG)。
  1. 妊娠の危険因子は遺残心室中隔欠損、中等度以上の肺動脈弁狭窄・逆流、中等度以上の大動脈弁逆流、上行大動脈拡張(40mm以上)、右室機能不全(心胸郭比60%以上)、左室機能不全(駆出率40%以下)、頻脈型不整脈の既往である[1](エビデンスランクOJG)。
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
小野博 : 特に申告事項無し[2021年]
監修:五十嵐隆 : 特に申告事項無し[2021年]

改訂のポイント:
  1. 定期レビューを行い、成人例の問題点(遺残病変、妊娠出産)を詳細に追加した(小児期の治療成績の向上により、成人手術後症例が増加したため)。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. 1671年にNiels Stensenにより最初に報告され、1888年にEtienne-Louis Fallotがその著書である“L’anaromie pathologique de la maladie bleu”に表記した[2][3]
  1. ファロー四徴という呼称は1924年にカナダ人の Maude Abbottによって名付けられた[2][3]
  1. 先天性心疾患の3~5%を占め、出生1,000に対し0.28の発生率である[4]
  1. 男女差はなく孤発例が多数を占め、同胞の再発率は3%といわれている[4]
  1. 22q11.2の微小欠失が、他の症候群が否定された本疾患の約6%に認められるとする報告もある[5]
  1. 四徴とは①心室中隔欠損、②大動脈騎乗、③右室流出路狭窄、④右室肥大――である。
  1. 漏斗部中隔が前方に偏位したことがその成因である。
  1. 1955年に最初の心内修復術が報告された[6]
  1. ファロー四徴症は小児慢性特定疾病の対象疾患に指定されている。家庭の収入などに応じて医療費が助成される(参照:小児慢性特定疾病センター
  1. ファロー四徴症は、指定難病であり、NYHA 心機能分類でⅡ度以上の場合などでは、申請し認定されると保険料の自己負担分の一部が公費負担として助成される。([平成27年7月施行])
  1.  難病法に基づく医療費助成制度 
問診・診察のポイント  
  1. 胎児心エコーによる胎児診断が可能である。

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文献 

著者: Christian Apitz, Gary D Webb, Andrew N Redington
雑誌名: Lancet. 2009 Oct 24;374(9699):1462-71. doi: 10.1016/S0140-6736(09)60657-7. Epub 2009 Aug 14.
Abstract/Text Tetralogy of Fallot is the most common form of cyanotic congenital heart disease, and one of the first to be successfully repaired by congenital heart surgeons. Since the first procedures in the 1950s, advances in the diagnosis, perioperative and surgical treatment, and postoperative care have been such that almost all those born with tetralogy of Fallot can now expect to survive to adulthood. The startling improvement in outcomes for babies born with congenital heart disease in general-and for those with tetralogy of Fallot in particular-is one of the success stories of modern medicine. Indeed, in many countries adults with tetralogy of Fallot outnumber children. Consequently, new issues have emerged, ranging from hitherto unpredicted medical complications to issues with training for caregivers and resource allocation for this population of survivors. Therefore, evolution of treatment, recognition of late complications, research on disease mechanisms and therapies-with feedback to changes in care of affected children born nowadays-are templates on which the timely discussion of organisation of care of those affected by congenital heart diseases from the fetus to the elderly can be based. Here, we focus on new developments in the understanding of the causes, diagnosis, early treatment, and late outcomes of tetralogy of Fallot, emphasising the continuum of multidisciplinary care that is necessary for best possible lifelong treatment of the 1% of the population born with congenital heart diseases.

PMID 19683809  Lancet. 2009 Oct 24;374(9699):1462-71. doi: 10.1016/S01・・・
著者: Richard Van Praagh
雑誌名: Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009;:19-38. doi: 10.1053/j.pcsu.2009.01.004.
Abstract/Text Stella Van Praagh, MD (1927-2006) of Children's Hospital Boston was one of the greatest pediatric cardiologists and pediatric cardiac pathologists of the 20th and early 21st centuries. Née Stella Zacharioudaki from Crete, Greece, in addition to her stellar professional attainments, she was also an outstanding cuisinière, hostess, linguist, philosopher, and philanthropist. In 1962, she married Richard Van Praagh, MD, beginning a life-long collaboration that was in every sense an affaire de coeur. They had three children and seven grandchildren. Dr Stella was the author of more than 110 scientific publications which helped to clarify the pathologic anatomy, the clinical and laboratory diagnosis, and often the surgical management of many different forms of congenital heart disease, including dextrocardia, single ventricle, truncus arteriosus, tetralogy of Fallot (TOF), transposition of the great arteries, double-outlet right ventricle, sinus venosus defect, anomalous pulmonary venous drainage, the heterotaxy syndromes with asplenia or polysplenia, juxtaposition of the atrial appendages, and apical muscular ventricular septal defect. In 1999, Dr Stella Van Praagh received the Distinguished Achievement Award of the Society for Cardiovascular Pathology, and in 2004, she was honored with the Paul Dudley White Award of the American Heart Association. Dr. Stella Van Praagh was that vanishingly rare combination of brilliant clinician, internationally renowned medical scientist, and deeply cultivated humanist. The anomaly now known as the TOF was first described by Niels Stensen in 1671, with other early reports by Edouard Sandifort (1777), William Hunter (1784), and many others. In 1888, Etienne-Louis Arthur Fallot published five serialized contributions in Marseille Médical concerning what he called the "blue malady," in which he described the now classical tetralogy of pulmonary outflow tract obstruction, ventricular septal defect, aortic overriding, and right ventricular hypertrophy. The other outstanding feature of Fallot's report was its emphasis on clinicopathologic correlation. In 1924, Maude Abbott coined the term "tetralogy of Fallot." In 1970, Van Praagh and colleagues presented the concept that the TOF is basically just one anomaly, a failure of normal expansile growth of the subpulmonary infundibulum and its sequelae. The anatomy of TOF is presented angiocardiographically, diagrammatically, and anatomically. A morphometric study of typical neonatal TOF is presented, based on 16 autopsied heart specimens with age-matched normal controls. The morphometric study documents that TOF is characterized by a low-volume subpulmonary infundibulum. The diagnostic and surgical significance of these findings is highlighted. Two rare and recently discovered forms of TOF are presented: tetralogy {S,D,I}, and tetralogy {I,D,S}. Because tetralogy {I,D,S} has atrioventricular discordance, in addition to a standard TOF repair, such patients also need an inverted (mirror-image) atrial switch operation (inverted Senning or inverted Mustard procedure). Because associated malformations can be very important to the surgical outcome of patients with tetralogy, the associated anomalies found in 100 randomly selected autopsied cases are presented.

PMID 19349011  Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 20・・・
著者: Elliot A Shinebourne, Sonya V Babu-Narayan, Julene S Carvalho
雑誌名: Heart. 2006 Sep;92(9):1353-9. doi: 10.1136/hrt.2005.061143.
Abstract/Text
PMID 16908723  Heart. 2006 Sep;92(9):1353-9. doi: 10.1136/hrt.2005.061・・・
著者: L Gioli-Pereira, A C Pereira, D Bergara, S Mesquita, A A Lopes, J E Krieger
雑誌名: Int J Cardiol. 2008 Jun 6;126(3):374-8. doi: 10.1016/j.ijcard.2007.04.043. Epub 2007 Jun 29.
Abstract/Text BACKGROUND: Tetralogy of Fallot (TOF) is a congenital conotruncal heart defect commonly found in DiGeorge (DGS) and velocardiofacial (VCFS) syndromes. The deletion of chromosome 22q11 has also been demonstrated in sporadic or familial cases of TOF. The aim of the present study was to investigate the frequency of del22q11 in patients with non-syndromic TOF seen at a tertiary Pediatric Cardiology care center.
METHOD: One hundred and twenty three non-syndromic TOF patients were selected and evaluated by history, physical examination and review of medical records. Venous blood was drawn for genomic DNA extraction after informed consent 22q11 microdeletion diagnosis was conducted through a standardized SNP genotyping assay and consecutive homozygosity mapping. Phenotype-genotype correlations regarding cardiac anatomy were conducted.
RESULTS: We evaluated 123 non-syndromic TOF patients for a 22q11 deletion. 105 (85.4%) patients presented pulmonary stenosis and 18 (14.6%) had pulmonary atresia. Eight patients (6.5%) were found to have a deletion. Of the deleted patients, three (37.5%) presented pulmonary atresia. We have verified a tendency towards a higher prevalence of pulmonary atresia when comparing TOF patients with and without 22q11 microdeletion.
CONCLUSIONS: 22q11.2 deletion in non-syndromic TOF patients is present in approximately 6% of patients. We suggest a tendency towards a higher prevalence of pulmonary atresia in non-syndromic TOF patients with 22q11 microdeletion. Molecular genetic screening of non-syndromic TOF patient may be important for the correct care of these patients and a more specific genetic diagnostic and counseling.

PMID 17604138  Int J Cardiol. 2008 Jun 6;126(3):374-8. doi: 10.1016/j.・・・
著者: C W LILLEHEI, M COHEN, H E WARDEN, R C READ, J B AUST, R A DEWALL, R L VARCO
雑誌名: Ann Surg. 1955 Sep;142(3):418-42.
Abstract/Text
PMID 13249340  Ann Surg. 1955 Sep;142(3):418-42.
著者: Anne S Bassett, Eva W C Chow, Janice Husted, Rosanna Weksberg, Oana Caluseriu, Gary D Webb, Michael A Gatzoulis
雑誌名: Am J Med Genet A. 2005 Nov 1;138(4):307-13. doi: 10.1002/ajmg.a.30984.
Abstract/Text 22q11 Deletion Syndrome (22q11DS) is a common microdeletion syndrome with multisystem expression. Phenotypic features vary with age, ascertainment, and assessment. We systematically assessed 78 adults (36 M, 42 F; mean age 31.5, SD 10.5 years) with a 22q11.2 deletion ascertained through an adult congenital cardiac clinic (n = 35), psychiatric-related sources (n = 39), or as affected parents of subjects (n = 4). We recorded the lifetime prevalence of features requiring attention, with 95% confidence intervals (CI) not overlapping zero. Subtle learning difficulties, hypernasality and facial gestalt were not included. We investigated ascertainment effects using non-overlapping subgroups ascertained with tetralogy of Fallot (n = 31) or schizophrenia (n = 31). Forty-three features met inclusion criteria and were present in 5% or more patients, including several of later onset (e.g., hypothyroidism, cholelithiasis). Number of features per patient (median 9, range 3-22) correlated with hospitalizations (P = 0.0002) and, when congenital features were excluded, with age (P = 0.02). Adjusting for ascertainment, 25.8% (95% CI, 9.5-42.1%) of patients had cardiac anomalies and 22.6% (95% CI, 7.0-38.2%) had schizophrenia. Ascertainment subgroups were otherwise similar in median number and prevalence of features. Non-characteristic features are common in 22q11DS. Adjusting for ascertainment effects is important. Many treatable conditions may be anticipated and features may accumulate over time. The results have implications for clinical assessment and management, genetic counseling and research into pathophysiological mechanisms.

Copyright 2005 Wiley-Liss, Inc
PMID 16208694  Am J Med Genet A. 2005 Nov 1;138(4):307-13. doi: 10.100・・・
著者: G S Van Arsdell, G S Maharaj, J Tom, V K Rao, J G Coles, R M Freedom, W G Williams, B W McCrindle
雑誌名: Circulation. 2000 Nov 7;102(19 Suppl 3):III123-9.
Abstract/Text BACKGROUND: Controversy regarding the timing for the repair of tetralogy of Fallot centers around initial palliation versus primary repair for the symptomatic neonate/young infant and the optimal age for repair of the asymptomatic child. We changed our approach from one of initial palliation in the infant to one of primary repair around the age of 6 months, or earlier if clinically indicated. We examined the effects of this change in protocol and age on outcomes.
METHODS AND RESULTS: The records of 227 consecutive children who had repair of isolated tetralogy of Fallot from January 1993 to June 1998 were reviewed. The median age of repair by year fell from 17 to 8 months (P:<0.01). The presence of a palliative shunt at the time of repair decreased from 38% to 0% (P:<0.01). Mortality (6 deaths, 2. 6%) improved with time (P:=0.02), with no mortality since the change in protocol (late 1995/early 1996). Multivariate analysis for physiological outcomes of time to lactate clearance, ventilation hours, and length of stay, but not death, demonstrated that an age <3 months was independently associated with prolongation of times (P:<0.03). Each of the deaths occurred with primary repair at an age >12 months. The best survival and physiological outcomes were achieved with primary repair in children aged 3 to 11 months.
CONCLUSIONS: On the basis of mortality and physiological outcomes, the optimal age for elective repair of tetralogy of Fallot is 3 to 11 months of age.

PMID 11082374  Circulation. 2000 Nov 7;102(19 Suppl 3):III123-9.
著者: Y Shimazaki, E H Blackstone, J W Kirklin
雑誌名: Thorac Cardiovasc Surg. 1984 Aug;32(4):257-9. doi: 10.1055/s-2007-1023399.
Abstract/Text The natural history of isolated congenital pulmonary valve incompetence has been determined by actuarial and parametric techniques from 72 cases collected from the literature. Symptoms developed within 20 years in 6% (CL 4% to 9%) of the patients and within 40 years in 29% (CL 22% to 36%). The hazard function of the development of symptoms demonstrates a risk increasing in time, particularly evident after 40 years. Death followed the appearance of symptoms after an average of 39 months in 3 patients who died. The surgical implications as regards the use of transannular patches and valveless extracardiac conduits are discussed.

PMID 6207619  Thorac Cardiovasc Surg. 1984 Aug;32(4):257-9. doi: 10.1・・・
著者: M A Gatzoulis, A L Clark, S Cullen, C G Newman, A N Redington
雑誌名: Circulation. 1995 Mar 15;91(6):1775-81.
Abstract/Text BACKGROUND: We have shown previously that transient right ventricular restriction after tetralogy of Fallot repair prolongs postoperative course. This is a prospective study of right ventricular diastolic performance in late follow-up patients.
METHODS AND RESULTS: We studied biventricular function, using Doppler echocardiographic examination. Pulmonary arterial, tricuspid, and mitral valves and superior vena cava Doppler spectrals were obtained in 41 patients (mean age, 28.8 years), 15 to 35 years (mean, 23.6) after complete repair of tetralogy of Fallot. Patients were considered to have evidence of right ventricular restriction if antegrade diastolic flow was detected in the main pulmonary artery, coinciding with atrial systole (A wave), throughout the respiratory cycle. Exercise function was measured by graded treadmill testing with respiratory mass spectrometry. Three patients were excluded because of pulmonary outflow obstruction (Doppler gradient > 40 mm Hg) or residual intracardiac shunts. Of the 38 patients, 37 were in sinus rhythm. Twenty (52.6%) had definite evidence of restriction with an A wave in the pulmonary artery, augmented during inspiration. In all 20 cases, there was superior vena caval flow reversal with atrial systole. Both inspiratory and expiratory transtricuspid E-wave deceleration time was significantly shorter in the restrictive group (P < .003 and P < .03, respectively). All patients had Doppler evidence of pulmonary regurgitation, but its duration was shorter in the restrictive group (P < .01) during inspiration. Cardiothoracic ratio was significantly lower in the restrictive group (P < .01), suggesting less severe pulmonary regurgitation. Both restrictive and nonrestrictive groups had reduced exercise MVO2 compared with healthy age- and sex-matched control subjects, but those with restrictive physiology had significantly better maximum oxygen uptake than the nonrestrictive group (P < .001).
CONCLUSIONS: Isolated right ventricular restriction late after tetralogy of Fallot repair is common. Although it reflects abnormal hemodynamics, the A wave contributes to forward pulmonary arterial flow and shortens the duration of pulmonary regurgitation. Consequently, there is less cardiomegaly and improved exercise performance in those patients.

PMID 7882487  Circulation. 1995 Mar 15;91(6):1775-81.
著者: Judith Therrien, Yves Provost, Naeem Merchant, William Williams, Jack Colman, Gary Webb
雑誌名: Am J Cardiol. 2005 Mar 15;95(6):779-82. doi: 10.1016/j.amjcard.2004.11.037.
Abstract/Text The timing of pulmonary valve replacement in adult patients with repaired tetralogy of Fallot remains controversial. A magnetic resonance imaging study in 17 adult patients with repaired tetralogy of Fallot reveals a statistically significant decrease in right ventricular (RV) volume (RV end-diastolic volume 163 +/- 34 to 107 +/- 26 ml/m2, p <0.001; RV end-systolic volume 109 +/- 27 to 69 +/- 22 ml/m2, p <0.001) at a mean follow-up of 21 months after pulmonary valve replacement; whereas RV systolic function remained unchanged (mean RV ejection fraction 32 +/- 7% to 34 +/- 10%, p = 0.12). In no patients with a RV end-diastolic volume >170 ml/m2 or a RV end-systolic volume >85 ml/m2 before pulmonary valve replacement were RV volumes "normalized" after surgery.

PMID 15757612  Am J Cardiol. 2005 Mar 15;95(6):779-82. doi: 10.1016/j.・・・
著者: Thomas Oosterhof, Alexander van Straten, Hubert W Vliegen, Folkert J Meijboom, Arie P J van Dijk, Anje M Spijkerboer, Berto J Bouma, Aeilko H Zwinderman, Mark G Hazekamp, Albert de Roos, Barbara J M Mulder
雑誌名: Circulation. 2007 Jul 31;116(5):545-51. doi: 10.1161/CIRCULATIONAHA.106.659664. Epub 2007 Jul 9.
Abstract/Text BACKGROUND: To facilitate the optimal timing of pulmonary valve replacement, we analyzed preoperative thresholds of right ventricular (RV) volumes above which no decrease or normalization of RV size takes place after surgery.
METHODS AND RESULTS: Between 1993 and 2006, 71 adult patients with corrected tetralogy of Fallot underwent pulmonary valve replacement in a nationwide, prospective follow-up study. Patients were evaluated with cardiovascular magnetic resonance both preoperatively and postoperatively. Changes in RV volumes were expressed as relative change from baseline. RV volumes decreased with a mean of 28%. RV ejection fraction did not change significantly after surgery (from 42+/-10% to 43+/-10%; P=0.34). Concomitant RV outflow tract reduction resulted in a 25% larger decrease of RV volumes. After correction for surgical RV outflow tract reduction, higher preoperative RV volumes (mL/m2) were independently associated with a larger decrease of RV volumes (RV end-diastolic volume: beta=0.41; P<0.001). Receiver operating characteristic analysis revealed a cutoff value of 160 mL/m2 for normalization of RV end-diastolic volume or 82 mL/m2 for RV end-systolic volume.
CONCLUSIONS: Overall, we could not find a threshold above which RV volumes did not decrease after surgery. Preoperative RV volumes were independently associated with RV remodeling and also when corrected for a surgical reduction of the RV outflow tract. However, normalization could be achieved when preoperative RV end-diastolic volume was <160 mL/m2 or RV end-systolic volume was <82 mL/m2.

PMID 17620511  Circulation. 2007 Jul 31;116(5):545-51. doi: 10.1161/CI・・・
著者: Luke J Burchill, Rachel M Wald, Louise Harris, Jack M Colman, Candice K Silversides
雑誌名: Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2011;14(1):92-7. doi: 10.1053/j.pcsu.2011.01.016.
Abstract/Text There is a growing population of young adults with tetralogy of Fallot. Although surgical approaches have evolved, many adults with repaired tetralogy of Fallot have been left with residual pulmonary regurgitation. Pulmonary regurgitation is an important contributor to a number of late complications including exercise limitations, right heart failure, arrhythmia, and sudden death. Because bioprosthetic valves are used in this population, clinicians must weigh the beneficial effects of pulmonary valve replacement against the associated risks, including subsequent re-operation. In this review, we will appraise the evidence supporting pulmonary valve replacement in the adult with repaired tetralogy of Fallot, as well as the optimal timing and mode of intervention.

Copyright © 2011 Elsevier Inc. All rights reserved.
PMID 21444054  Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 20・・・
著者: M A Gatzoulis, J A Till, J Somerville, A N Redington
雑誌名: Circulation. 1995 Jul 15;92(2):231-7.
Abstract/Text BACKGROUND: Life-threatening ventricular arrhythmia and sudden death remain serious late complications after tetralogy of Fallot repair. Nevertheless, there remains no clear way of predicting which patients are at risk.
METHODS AND RESULTS: The study population included a total of 178 adult survivors (mean follow-up, 21.4 years) of tetralogy of Fallot repair who were currently attending our clinic. Mechano-electrical relations were sought in 41 of the patients (mean follow-up, 23.6 years) who were operated on by one surgeon and who were prospectively studied with a 12-lead ECG, chest radiography, and two-dimensional and Doppler echocardiography. Nine patients (mean follow-up, 17 years) from the total group of 178 were identified as having had sustained ventricular tachycardia (8 with near-miss sudden death), and their ECGs, Holter monitor readings, electrophysiological studies, and chest radiographs were reviewed. The case notes of an additional 4 patients with postoperative sudden cardiac death also were available for review. QRS duration in the 41 patients in whom mechanoelectrical interaction was sought ranged between 90 and 200 milliseconds and correlated with cardiothoracic ratio (CTR) on chest radiography (r = .64, P < .001) and with right ventricular size on echocardiography (r = .43, P < .02). Twenty of the 41 patients had restrictive right ventricular Doppler physiology (reduced ventricular compliance) with mean QRS duration of 129.3 +/- 20 milliseconds and mean CTR of 0.51 +/- 0.03. The remaining 21 patients with no evidence of right ventricular restriction had prolonged QRS duration of 157.5 +/- 13.2 milliseconds (P < .001) and CTR of 0.55 +/- 0.04 (P < .04) compared with the restrictive. In the 9 patients with ventricular tachycardia, the QRS duration ranged from 180 to 230 milliseconds (mean, 198.9 +/- 17.6 milliseconds), and the CTR ranged from 0.54 to 0.9 (mean, 0.67 +/- 0.12) (P < .0001 and P < .01, respectively, compared with patients without life-threatening arrhythmias). All patients with documented sustained ventricular tachycardia and the 4 patients with sudden death had a QRS duration of > or = 180 milliseconds (100% sensitivity).
CONCLUSIONS: Chronic right ventricular volume overload after tetralogy of Fallot repair is related to diastolic function and correlated with QRS prolongation. The risk of symptomatic arrhythmia is high when marked right ventricular enlargement and QRS prolongation develop. A QRS duration on the resting ECG of > or = 180 milliseconds is the most sensitive predictor of life-threatening ventricular arrhythmias yet described.

PMID 7600655  Circulation. 1995 Jul 15;92(2):231-7.
著者: Thomas Oosterhof, Hubert W Vliegen, Folkert J Meijboom, Aeilko H Zwinderman, Berto Bouma, Barbara J M Mulder
雑誌名: Heart. 2007 Apr;93(4):506-9. doi: 10.1136/hrt.2006.094169. Epub 2006 Oct 25.
Abstract/Text OBJECTIVE: To analyse the long-term course of QRS duration after pulmonary valve replacement in patients with a previous correction for tetralogy of Fallot.
SETTING: Tertiary referral centres.
METHODS: In a retrospective study, 99 adult patients with tetralogy of Fallot, who had undergone a first pulmonary valve replacement late after initial total correction, were identified from the CONCOR (CONgenital CORvitia) registry. Computer-generated QRS durations were obtained from 12-lead electrocardiogram ECG reports in the medical records. A mixed linear regression model was used to analyse the course of QRS duration over time and to identify risk factors for increase in QRS duration over time. Composite end point was created from sudden cardiac death, ventricular tachycardia or implantable cardioverter-defibrillator discharge.
RESULTS: In total, 99 patients (57% men, mean (SD) age at pulmonary valve replacement 29 (11) years) with a median follow-up of 4.9 (0.1-16) years were analysed. In patients with preoperative QRS <120 ms, surgery caused no significant change in QRS duration (increase 1.3 (7.9) ms; p = 0.65), and after surgery, QRS duration remained stable over time (increase 0.0064 (0.059) ms/year; p = 0.98). By contrast, in patients with a preoperative QRS of 150-180 ms or QRS > or =180 ms, surgery resulted in QRS shortening (mean decrease 9.9 (SE 4.3) ms, p = 0.021, and 12.2 (SE 2) ms; p<0.001, respectively). During follow-up, a QRS widening 1.1(1.3) ms/year (p<0.001) in both groups was observed. In patients with a preoperative QRS > or =180 ms, no significant difference was observed in the number of patients reaching the composite end point compared with patients with a preoperative QRS of 150-180 ms (25% vs 7%; p = 0.08). However, the former more often reached QRS > or =180 ms again after surgery compared with the latter (53% vs 13%; p = 0.02, respectively). None of the patients with a preoperative QRS > or =180 ms died during follow-up.
CONCLUSION: In our study, we observed a decrease in QRS duration directly after surgery, followed by a steady increase, in patients with a preoperative QRS >150 ms. The beneficial effect of pulmonary valve replacement on QRS duration was transient. The risk of developing ventricular arrhythmias after surgery was substantial when preoperative QRS was > or =180 ms, but mortality remained low.

PMID 17065183  Heart. 2007 Apr;93(4):506-9. doi: 10.1136/hrt.2006.0941・・・

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