Late Aortic Root Dilatation in Tetralogy of Fallot may be Prevented by Early Repair in Infancy

The objectives of this study were to examine the relative contributions of development and hemodynamics in aortic root dilatation of tetralogy of Fallot, to assess the impact of systemic to pulmonary artery shunt on aortic annular size, and to seek any relationship between the timing of corrective surgery and subsequent aortic root size. We performed a retrospective analytical study at a tertiary referral center of M-mode and two-dimensionol aortic root measurements in children with tetralogy of Fallot prior to any surgical or palliative intervention, after insertion of a surgical shunt, and on intermediate and long-term follow-up post-repair. The main outcome measures were aortic root diameter prior to correction, after palliative shunt insertion, and after definitive repair. The study found that the aortic root is enlarged in unrepaired tetralogy of Fallot irrespective of age, increased in the first 3–6 months after palliative shunt surgery, and persisted into adulthood in those repaired after 1 year of age. Age-corrected aortic root dimensions normalized by midchildhood in those who were repaired in infancy. Preexisting aortic root dilatation normalizes by 7 years of age in patients with tetralogy of Fallot who have been repaired in infancy, whereas it persists into adulthood in the group repaired postinfancy.     

Tetralogy of Fallot with Aortic Stenosis: Repair Without Aortic Valve Intervention

A 14-day-old boy with tetralogy of Fallot (ToF), ductus-dependent pulmonary circulation, and significant aortic valve stenosis (AVS) underwent primary repair of ToF without aortic valve intervention. The pressure gradient through the aortic valve decreased spontaneously after the operation. This may have been due to the reduction of blood flow through the aortic valve postoperatively. For patients with ToF, AVS, and ductus-dependent circulation, ToF repair alone may alleviate the severity of the AVS.     

Reconstruction of an Isolated Left Subclavian Artery in a Patient with a Right Aortic Arch and Tetralogy of Fallot

An isolated left subclavian artery (ILSA) is very rarely seen in patients with a right aortic arch. This report describes the case of a 2-year-old boy who underwent ILSA reconstruction during repair of the associated tetralogy of Fallot to prevent future subclavian steal syndrome after surgery.     

Course of Right and Left Ventricular Function in Patients with Pulmonary Insufficiency After Repair of Tetralogy of Fallot

Surgical repair of tetralogy of Fallot (TOF) frequently results in pulmonary valve insufficiency. Nevertheless, no serial information is available on the long-term impact of the valvular insufficiency on right and left ventricular function. Right and left ventricular ejection fraction was measured serially by radionuclide angiocardiography in 21 patients with at least moderate pulmonary insufficiency after repair of TOF. A baseline study was obtained an average of 1.2 years after repair, and a follow-up study was performed an average of 10.2 years after surgery. Changes in ventricular function over time and deviations from the normal range were analyzed. At baseline evaluation the mean right ventricular ejection fraction (RVEF; 0.52 ± 0.10) and left ventricular ejection fraction (LVEF; 0.68 ± 0.10) were normal. At the time of follow-up the mean RVEF had significantly decreased to 0.45 ± 0.09 (p < 0.01). The mean LVEF had decreased to 0.60 ± 0.11 (p < 0.02). This change was independent of the RVEF (r=−0.13). Eleven patients (52%) had an abnormal RVEF or LVEF at follow-up. Nineteen patients (90%) showed a decrease of 0.05 or more in RVEF, LVEF, or both between studies. These data suggest a negative impact of long-standing pulmonary insufficiency on right and left ventricular systolic function after repair of TOF. Therefore, continued surveillance of biventricular function in this patient population appears warranted.     

Tetralogy of fallot and coarctation of the aorta: A rare combination and its clinical implications

Summary A neonate with tetralogy of Fallot and aortic coarctation is described. The clinical course was remarkable, with early congestive heart failure followed by rapidly progressive left ventricular myocardial thickening and the presentation of hypercyanotic spells. Primary repair of tetralogy without correction of coarctation was attempted unsuccessfully at 1 month of age. Death followed postoperative bradycardia, hypotension, and low cardiac output. Fetal and neonatal hemodynamics and the clinical implication of this rare combination of defects are discussed. Initial surgical intervention should relieve left-sided obstruction.     

Role of transesophageal echocardiography in the management of aortic-left ventricular tunnel

A 10-year-old patient has been followed for 10 years after repair of an aortic-left ventricular tunnel (ALVT) for residual aortic regurgitation and significant recurrent leak through the ALVT. Transesophageal echocardiography (TEE) was done prior to surgery and intraoperatively to (1) clarify the precise anatomy of the ALVT, (2) evaluate the degree of aortic regurgitation, and (3) assess the repair. Delineation of the anatomy of the ALVT and assessment of the residual aortic insufficiency helped with the intraoperative decisionmaking. We describe the TEE findings of a residual ALVT in this patient and discuss the role of TEE in managing the lesion.     

Successful Staged Neonatal Repair of Tetralogy of Fallot with Long-Segment Hypoplasia of the Aorta

We describe an extremely rare combination of tetralogy of Fallot (TOF), right-sided cervical aortic arch with long-segment hypoplasia, and other vascular anomalies. A two-stage surgical approach included aortic arch reconstruction followed by right ventricular muscle bundle division and ventricular septal defect closure a few weeks later. The initial clinical presentation, perioperative course, and imaging studies are presented along with a review of the relevant literature. This is the first report of successful neonatal repair of TOF with long-segment hypoplasia of the aorta.     

Life insurance for children with cardiovascular disease

To determine the life insurability of young people with cardiovascular disease, we sent questionnaires to 99 life insurance companies concerning 18 congenital defects, rheumatic heart disease, and four dysrhythmias. We received 50 responses (50%) from companies whose sales make up 41% of the life insurance market. The concensus of insurability for the defects listed was: standard rates--mild pulmonic stenosis, rheumatic fever without carditis, mitral valve prolapse without regugitation, and the following postoperative lesions: patent ductus arteriosus, atrial septal defect, pulmonic stenosis, ventricular septal defect; uninsurable--most unoperated lesions, postoperative lesions with complex dysrrhythmias, severe aortic insufficiency, idiopathic hypertrophic subaortic stenosis, Ebstein's anomaly, truncus arteriosus, tricuspid atresia; insurable at increased rates--most other defects, including dextrotransposition of the great vessels, postoperative aortic stenosis, mild aortic insufficiency, postoperative coarctation of aorta, postoperative tetralogy of Fallot, and small ventricular septal defect. We conclude that life insurance is available to many children with cardiovascular disease, including most postoperative patients. Whether the increased rates requested for some defects are prohibitive is a matter to be decided by each family.     

Tetralogy of Fallot with Congenital Aortic Valvar Stenosis: The Tetralogy–Truncus Interrelationship

Two rare patients are reported with tetralogy of Fallot and congenital aortic valvar stenosis. The anatomic and developmental interrelationship between tetralogy of Fallot and truncus arteriosus is summarized. A study of 100 randomly selected postmortem cases of tetralogy revealed aortic valve pathology in 8%, myxomatous aortic valve leaflets without stenosis in 4%, bicuspid aortic valves without stenosis in 3%, and congenital aortic valvar stenosis in 1%. The frequency of systemic semilunar valve pathology in truncus was much higher (66%): moderate to marked myxomatous change in 44%, mild myxomatous change in 22%, truncal valvar stenosis in 11%, and truncal valvar regurgitation in 15%. Being aware of the tetralogy–truncus interrelationship and knowing that myxomatous aortic valves are prone to premature calcific aortic stenosis and/or regurgitation, physicians should follow the aortic valves of surgically repaired patients with tetralogy of Fallot and truncus arteriosus long term with great care. Timely aortic valvuloplasty or replacement may well prove life-saving in such patients.     

Ruptured Sinus of Valsalva Found Incidentally in a Patient with Tetralogy of Fallot

Ruptured sinus Valsalva aneurysm (SVA) in a patient with tetralogy of Fallot is a very rare cardiac complication. This report describes the case of a 10-year-old Hispanic girl who presented to her cardiologist for a routine transthoracic echocardiography (TTE), which showed a ruptured sinus of Valsalva, with left-to-right shunting noted from the right cusp to the right ventricular outflow tract and pulmonic insufficiency. The patient underwent right and left heart catheterization with transesophageal echocardiography guidance and closure of the ruptured sinus of Valsalva with an Amplatzer muscular ventricular septal defect (VSD) device. Later that day, after the procedure, it was noted that the patient had a recurrence of continuous murmur. The TTE did not visualize the Amplatzer VSD device, and chest X-ray showed that the device had embolized to the right pulmonary artery. The device was successfully retrieved percutaneously. The patient returned 2 weeks later for surgical repair of a ruptured SVA and pulmonary valve replacement, which went well. This case report is the first to describe congenital rupture of a Valsalva aneurysm in a patient with tetralogy of Fallot. The conventional treatment for a ruptured sinus of Valsalva is surgical, although transcatheter approaches have been used increasingly in recent years.     

Rheumatic Fever in Children: A 15-Year Experience in a Developing Country

Clinical data from 91 patients with rheumatic fever (RF), who were hospitalized at a tertiary hospital in Lebanon between 1980 and 1995, were reviewed retrospectively. Age on hospitalization was 11.1 ± 2.9 years (mean ± SD, range 3–17 years). Nineteen patients were <6 years of age. Manifestations included carditis (93%), arthritis (39%), Sydenham's chorea (2%), erythema marginatum (4%), subcutaneous nodules (1%), fever (62%), arthralgia (55%), and acute congestive heart failure (CHF) on initial presentation (44%). Pericardial effusion occurred in 11%. There was positive family history of RF in 14%. Mitral insufficiency and aortic insufficiency occurred in 67 and 35%, respectively. Both mitral and aortic valves were involved in 30% of cases. Tricuspid insufficiency developed in 3% and pulmonary insufficiency in 1%. Mitral stenosis developed in 19%. Twenty-eight patients underwent surgical intervention: mitral valve repair and commissurotomy in 9/91 (10%), mitral valve replacement in 18/91 (20%), and aortic valve replacement in 9/91 (10%). Overall mortality was 12%: 5 following surgical intervention (3 after mitral valve surgery and 2 after mitral and aortic valve surgery). All patients that died had CHF on initial presentation (p= 0.006). This study includes hospitalized patients with predominant rheumatic heart disease. Initial presentation with CHF is a risk factor for surgical intervention and mortality. A significant high surgical intervention rate is noted that is probably related to the nature of the selected group studied. This study emphasizes the significant morbidity and death in patients with RF and carditis.     

Left Hemitruncus Associated with Tetralogy of Fallot: Fetal Diagnosis and Postnatal Echocardiographic and Cardiac Computed Tomographic Confirmation

Anomalous origin of one pulmonary artery from the aorta, or hemitruncus, is a rare cardiac malformation. We report a case of left hemitruncus (aortic origin of the left pulmonary artery) associated with tetralogy of Fallot diagnosed in utero. To the authors’ knowledge, this is the first such case diagnosed by fetal echocardiography to be described in the literature. The condition was documented by postnatal echocardiogram and cardiac computed tomography. Prompt recognition of this lesion is essential for early repair to improve outcome.     

Health-Related Quality of Life Compared With Cardiopulmonary Exercise Testing at the Midterm Follow-up Visit After Tetralogy of Fallot Repair: A Study of the German Competence Network for Congenital Heart Defects

This nationwide study aimed to evaluate health-related quality of life (QoL) experienced by children after tetralogy of Fallot repair and to compare self-reported physical ability with objective exercise performance. This prospective nonrandomized, government-funded multicenter study enrolled 168 patients (70 girls; ages 8–16 years) after tetralogy of Fallot repair at eight German heart centers. Health-related QoL was analyzed by the self-reported KINDL-R quality-of-life questionnaire. The patients’ actual exercise capacity was evaluated by a cardiopulmonary exercise test. Health-related QoL and cardiopulmonary exercise capacity were compared with those of an age-matched German standard population. Correlation of health-related QoL with self-estimated physical rating and cardiopulmonary exercise capacity were analyzed. Health-related QoL in children and adolescents after tetralogy of Fallot repair is without limitation. Compared with the standard population, all the items evaluated by the KINDL-R questionnaire showed better or similar values, whereas objective exercise capacity compared with that of the standard population was impaired. Peak oxygen uptake correlated significantly with the physical well-being (p = 0.002) and the total score (p = 0.01) of the KINDL-R questionnaire. Health-related QoL experienced by children and adolescents after tetralogy of Fallot repair is comparable with that of the healthy standard population. However, closer inspection shows that self-estimated physical functioning is significantly overestimated compared with actual exercise capacity. Quality-of-life instruments and exercise tests, therefore, should be used in a complementary manner with children to avoid eventually fatal misinterpretation of patient-estimated physical ability.     

婴幼儿法乐四联症手术治疗近期疗效的影响因素

目的 探讨影响婴幼儿法乐四联症手术治疗近期疗效的各相关因素.方法 将2003年8月至2011年2月经作者一期手术纠治的117例年龄≤3岁的法乐四联症患儿分为疗效良好、疗效较差两组.分析手术时患儿年龄、体重、术前HCT值、McGoon指数、EDVI、主动脉阻断时间、转流时间、室间隔缺损大小、升主动脉与肺动脉干直径比、...     

Surgical management in tetralogy of Fallot and vascular ring

Summary The management of three infants born with a combination of tetralogy of Fallot and a vascular ring causing tracheoesophageal compression is described. There was a double aortic arch in two patients and an aberrant left subclavian artery with left ligamentum arteriosum and right aortic arch in one. Single-stage corrective surgery of both lesions during infancy, performed under profound hypothermia and circulatory arrest, was successful. In our opinion, this is the treatment of choice, when the anatomy of the tetralogy is favorable for primary correction.     

Pulmonary insufficiency in childhood. Contribution to diastolic murmurs (author's transl)

Diastolic murmurs in childhood are in most cases due to pulmonary insufficiency. They may be functional (Graham-Steel-murmurs, for instance with idiopathic pulmonary ectasis or pulmonary hypertension), but are more and more the symptom of postoperative pulmonary insufficiency. Diastolic murmurs of pulmonary insufficiency were found after operations of valvular and infundibular pulmonary stenosis in 92,5% of the cases (40 children) and after total correction of tetralogy of Fallot in 89% of 27 children. The diastolic murmur of pulmonary insufficiency is very good recorded in phonocardiography; with postoperative heart catheterisations and angiocardiography there is good estimation of the degree of the pulmonary insufficiency. There is no doubt about the curative effort of operations in these congenital heart defects, and postoperative pulmonary insufficiency does not impair the operative improvement in childhood. Till now long term observations of these cases up to late adulthood cannot be possible; regular pediatric cardiologic examinations of these operated children are mandatory.     

Velocity of Oxygen Uptake Response at the Onset of Exercise: A Comparison Between Children After Cardiac Surgery and Healthy Boys

A comparison was carried out concerning maximal oxygen uptake, oxygen uptake adjustment at the onset of high-intensity exercise, and maximal blood lactate between 10 healthy prepubertal boys and 35 children after repair of cardiac malformations or after Fontan operation. Mean maximal oxygen uptake (VO₂) was moderately reduced in children after repair of tetralogy of Fallot or after Mustard or Senning operations and severely reduced after Fontan operations. Conversely, mean half-time of VO₂ response was moderately prolonged in children after repair of tetralogy of Fallot or after Senning and Mustard operations and considerably prolonged after Fontan operations. According to our results unfavorable kinetics of VO₂ response to physical exercise are present in addition to reduced aerobic power in many of the operated children. Besides being less qualified for endurance performance, these children are also less prepared for short, high-intensity exercise.     

婴幼儿法洛四联症根治术85例

目的探讨经主肺动脉和右心房径路手术,根治婴幼儿法洛四联症（TOF）的适应证和临床疗效。方法对2008年1月至2012年12月我们收治的85例TOF患儿,采取经主肺动脉和右心房径路施行TOF根治术,占同期TOF手术的72．6％（85／117）。结果85例患儿均存活。术后均采用小剂量多巴胺辅助循环。随访2～60个月,无死亡病例。无明显肺动脉瓣反流,心功能良好。结论肺动脉瓣环发育良好的TOF婴幼儿可经主肺动脉和右心房径路行根治术,该术式肺动脉瓣完整,无右心室切口,右心功能良好,围手术期并发症少,近中期随访疗效良好。     

Surgical treatment of congenital heart malformations. Indications and surgical management (author's transl)]

Congenital malformations of the heart have to be expected in approximately 0,8% of all newborns. The majority of them needs surgical treatment and can be corrected anatomically with a low operative risk (atrial and ventricular septal defects, patent ductus arteriosus, coarctation, valvular aortic and pulmonary stenosis etc.). Surgical repair of complex anomalies includes either palliative or functionally corrective procedures and has a much higher operative and early postoperative mortality (transposition of the great arteries, tetralogy of Fallot, tricuspid atresia etc.). Surgery becomes urgent in babies suffering from congestive heart failure and/or progressive severe hypoxia during the first months of life. Early total surgical repair or primary palliation and two-stage correction depends on the kind of anomaly and has to be selected individually. Hemodynamical classification, general distribution and surgical considerations of the most common congenital malformations of the heart are described.     

Tetralogy of Fallot.

Tetralogy of Fallot is the most common malformation of children born with cyanotic heart disease, with an incidence of approximately 10 per cent of congenital heart disease. There can be a wide spectrum as to the severity of the anatomic defects, which include ventricular septal defect, aortic override, right ventricular outflow tract obstruction, and right ventricular hypertrophy. Cyanosis may vary from mild to severe, and patients may present as newborns or, more commonly, young infants. Infants with classic tetralogy of Fallot and stable anatomy should undergo primary complete intracardiac repair. The overall hospital mortality is approximately 3 to 5 per cent, with most patients who survive having an excellent clinical and hemodynamic result.