Tetralogy of Fallot: Yesterday and Today

Starr, Joanne P.

doi:10.1007/s00268-009-0296-8

Cited by 126 publications

(98 citation statements)

References 96 publications

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“…The risk of death is greatest in the first year of life. Major causes of death in surgically untreated patients include hypoxic spells (62%), cerebrovascular accidents (17%), and brain abscesses (13%) [1,3]. There are many conflicts about the optimum time and appropriate procedure for symptomatic patients with TOF.…”

Section: Discussionmentioning

confidence: 99%

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Preoperative Pulmonary Valvuloplasty inTetralogy of Fallot with Right-To-Left Shunt

Buğan¹

2016

Ann Clin Anal Med

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ÖzetFallot tetralojisi en sık saptanan siyanotik konjenital kalp hastalığıdır ve sağ ventrikül çıkış yolu obstrüksiyonu, ventriküler septal defekt, ata binen aorta ve sağ ventrikül hipertrofisi ile karakterizedir. Sağ ventrikül çıkış yolu obstrüksiyonu ve ventriküler septal defekt, sendromun majör klinik parçalarıdır. Çoğu hastaya düzel-tici bir operasyon uygulanmasına rağmen, Fallot tetralojili hastaların önemli olan az bir kısmına preoperatif palyatif bir işlem uygulanır. Burada biz sağdan-sola şant nedeniyle, düzeltici cerrahi için ameliyat edilemez olarak düşünülen Fallot tetralojili 19 yaşında bir bayan hastada, pulmoner darlığın başarılı palyatif perküta-nöz balon valvüloplastisini sunuyoruz. Anahtar KelimelerBalon Valvüloplasti; Sağ-Sol; Şant; Fallot Tetralojisi AbstractTetralogy of Fallot is the most common cyanotic congenital heart disease and characterized by right ventricular outflow tract obstruction, ventricular septal defect, overriding aorta, and right ventricular hypertrophy. Right ventricular outflow tract obstruction and ventricular septal defect are the major clinical components of the syndrome. Although most have undergone a corrective operation, an important minority of patients with tetralogy of Fallot have had a preoperative palliative procedure. Herein we reported a succesful palliative percutaneous balloon valvuloplasty of pulmonary stenosis at an 19-year-old female patient with tetralogy of Fallot who was considered as inoperable for corrective surgery due to right -to-left shunt.

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Section: Discussionmentioning

confidence: 99%

“…There is a wide clinical aspect of disease in tetralogy of Fallot (TOF), depending on the severity and localization of the right ventricular outflow truct obstruction (RVOTO) [1,2]. The ventricular septal defect (VSD) is generally large and non-restrictive.…”

Section: Introductionmentioning

confidence: 99%

Preoperative Pulmonary Valvuloplasty inTetralogy of Fallot with Right-To-Left Shunt

Buğan¹

2016

Ann Clin Anal Med

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“…2 Tetralogy of Fallot accounts for 7-10% of all congenital cardiac defects. 6 It consists of a VSD, an overriding aorta, and right ventricular hypertrophy resulting from right ventricular outflow tract obstruction (RVOTO) that favours a right-to-left shunt. Intracardiac pressures tend to equalize across the VSD, and cyanosis occurs due to shunting at a ventricular level and a reduction of blood volume in the pulmonary vasculature from the RVOTO.…”

Section: Discussionmentioning

confidence: 99%

Cardiac output monitoring during Cesarean delivery in a patient with palliated tetralogy of Fallot

Clivatti

Smith

Sermer

et al. 2012

Can J Anesth/J Can Anesth

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Purpose Tetralogy of Fallot (TOF) is one of the most common causes of cyanotic congenital heart disease. The anesthetic management of parturients with uncorrected TOF is challenging and controversial, especially for Cesarean delivery (CD). We describe the use of noninvasive cardiac output (CO) monitoring to assist the management of CD for a woman with palliated TOF under general anesthesia. Clinical features A 34-yr-old woman presented for elective CD at 38 weeks gestation. Having been born with TOF, she underwent a modified Blalock-Taussig shunt at six years of age, followed nine years later by creation of an aortopulmonary connection. The patient's functional status was New York Heart Association class I despite evident central cyanosis. A CD was performed under general anesthesia. Fentanyl, etomidate, and succinylcholine were utilized for induction, and intrathecal morphine was administered for postoperative pain control. The baseline CO (7.2 LÁmin -1 ), blood pressure (156/74 mmHg), heart rate (74 beatsÁmin -1 ), and total peripheral resistance (1,059 dynesÁsec -1 Ácm -5 ) remained stable throughout the procedure. Maintenance anesthesia consisted of rocuronium, sevoflurane, and an oxygen/nitrous oxide mixture. Upon delivery, an infusion of oxytocin combined with ergometrine was administered. Hemodynamic parameters remained stable and no vasopressor was required. Conclusion Balanced general anesthesia and careful titration of uterotonic agents provided stable hemodynamic conditions during CD in a patient with a palliated TOF, as assessed by a continuous noninvasive CO monitor. Noninvasive CO monitoring may improve our understanding of the hemodynamic implications of various anesthetic techniques for CD in cardiac patients. RésuméObjectif La te´tralogie de Fallot (TF) est l'une des causes les plus fre´quentes de cardiopathie conge´nitale cyanosante. La prise en charge anesthe´sique des parturientes ayant une TF non corrige´e est controverse´e et rele`ve du de´fi, en particulier en cas d'accouchement par ce´sarienne. Nous de´crivons l'utilisation d'un monitorage non invasif du de´bit cardiaque (DC) pour aider la prise en charge de la ce´sarienne chez une femme ayant une TF ope´re´e sous anesthe´sie ge´ne´rale. Caractéristiques cliniques Une femme aˆge´e de 34 ans se pre´sente pour une ce´sarienne programme´e à 38 semaines de grossesse. Elle est ne´e avec une TF et a subi une intervention modifie´e de Blalock-Taussig a`l'aˆge de six ans, puis neuf ans plus tard la cre´ation d'une connexion aorto-pulmonaire. Le statut fonctionnel de la patiente

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“…Complete repair can be performed with a low mortality risk (<2 %) (Starr 2010). Early outcome can be determined by right ventricle function in the immediate post-operative period.…”

Section: Introductionmentioning

confidence: 99%

Heat shock protein 27 is increased in cyanotic tetralogy of Fallot myocardium and is associated with improved cardiac output and contraction

Walker¹,

Danton

Peng

et al. 2013

Cell Stress and Chaperones

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Tetralogy of Fallot (TOF) is a congenital heart condition in which the right ventricle is exposed to cyanosis and pressure overload. Patients have an increased risk of right ventricle dysfunction following corrective surgery. Whether the cyanotic myocardium is less tolerant of injury compared to non-cyanotic is unclear. Heat shock proteins (HSPs) protect against cellular stresses. The aim of this study was to examine HSP 27 expression in the right ventricle resected from TOF patients and determine its relationship with right ventricle function and clinical outcome. Ten cyanotic and ten noncyanotic patients were studied. Western blotting was used to quantify HSP 27 in resected myocardium at (1) baseline (first 15 min of aortic cross clamp and closest representation of preoperative status) and (2) after 15 min during ischemia until surgery was complete. The cyanotic group had significantly increased haematocrit, lower O 2 saturation, thicker interventricular septal wall thickness and released more troponin-I on post-operative day 1 (p<0.05). HSP 27 expression was significantly increased in the <15 min cyanotic compared to the <15 min non-cyanotic group (p00.03). In the cyanotic group, baseline HSP 27 expression also significantly correlated with oxygen extraction ratio (p00.028), post-operative basal septal velocity (p00.036) and mixed venous oxygen saturation (p00.02), markers of improved cardiac output/contraction. Increased HSP 27 expression and associated improved right ventricle function and systemic perfusion supports a cardioprotective effect of HSP 27 in cyanotic TOF.

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Tetralogy of Fallot: Yesterday and Today

Cited by 126 publications

References 96 publications

Preoperative Pulmonary Valvuloplasty inTetralogy of Fallot with Right-To-Left Shunt

Preoperative Pulmonary Valvuloplasty inTetralogy of Fallot with Right-To-Left Shunt

Cardiac output monitoring during Cesarean delivery in a patient with palliated tetralogy of Fallot

Heat shock protein 27 is increased in cyanotic tetralogy of Fallot myocardium and is associated with improved cardiac output and contraction

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