Moderne Therapiestrategien bei Fallot-Tetralogie

Tetralogy of Fallot is one the most common congenital heart diseases. Currently, early primary correction in infancy is the procedure of choice which can be successfully performed even in symptomatic newborns. Palliative procedures are controversially discussed and widely avoided; however, palliation may still be indicated in high-risk patients. In addition to surgical procedures palliative interventional techniques have been developed. Currently, surgical correction is preferentially performed via the transatrial approach, if necessary in combination with a limited right ventriculotomy. Transannular enlargement of the right ventricular outflow tract should be avoided if possible. The prevention of pulmonary valve regurgitation is a major concern because of its deleterious effects on right ventricular function and its relevance on long-term morbidity. Currently developed techniques, such as interventional pulmonary valve implantation and tissue engineered heart valves may reduce the risk and need for re-operations in the future.     

Double-barrel right ventricular outflow: tetralogy of fallot annulus preservation technique

In patients with tetralogy of Fallot (TOF) repair and a borderline pulmonary valve annulus (PVA) size, surgical repair often necessitates a transannular incision and subsequent placement of a patch with or without a monocusp or, alternatively, a right ventricle-to-pulmonary artery conduit. We discuss here a technique in which the pulmonary valve annulus can be safely preserved, with infrequent postoperative issues as well as the potential for less incidence of right ventricular outflow intervention in the long term.     

Tetralogy of Fallot: to patch or not to patch?

Angiograms of 11 patients with Tetralogy of Fallot were reviewed before and after total repair, and analyzed with respect to growth of the pulmonary valve annulus. At the operation none of the patients was more than 5-years-old, none received a transannular patch and all were asymptomatic at the last visit. The radiologic diameter of the pulmonary valve annulus was compared to the descending thoracic aorta at the level of the diaphragm. Preoperative ratio averaged 1.09 +/- 0.22 and increased postoperatively to 1.35 +/- 0.20 (p less than 0.02). A "catch-up" growth of small annuli could be demonstrated.     

Valve‐sparing repair with skeletonization of the pulmonary annulus for tetralogy of Fallot

Pulmonary valve preservation in tetralogy of Fallot (TOF) repair is one of the most challenging issues. Herein, we describe a novel valve‐sparing technique for TOF repair that primarily consists of skeletonization of the anterior part of the pulmonary annulus and gentle dilatation by preserving the pulmonary valve and annulus integrity. With encouraging early results, this technique is suggested to prevent severe pulmonary regurgitation and provide acceptable relief of pulmonary stenosis in patients with TOF.     

Comparison of right ventricular function between prevention and enlargement of pulmonary valve annulus after repair of tetralogy of Fallot; mid-term results

Total of 41 patients with tetralogy of Fallot (TOF) who underwent intracardiac repair from 1993 to 1998 were divided into 2 groups: preservation (n = 14) or enlargement (n = 27) of the pulmonary valve annulus. The procedure was decided on the Z value of the annular size: above or under -2 SD of the standard value. Although postoperative right ventricular (RV) diastolic volume (RVEDV) and cardiothoracic ratio (CTR) were larger than the preservation group and pulmonary regurgitation (PR) existed in the enlargement group, RV pressure was decreased and central venous pressure (CVP) was low and RV contraction was preserved. The exercise capacity was also good and no significant arrhythmia was recognized. Our mid-term results showed that appropriate enlargement of the pulmonary valve annulus preserved good RV function in patients with TOF.     

First pulmonary valve replacement with Freedom Solo stentless valve in an adolescent. A case report

Tetralogy of Fallot (TOF) is a common form of cyanotic heart disease. Complete surgical correction in younger age group offers good long-term results with reasonable morbidity and improved prognosis in patients with TOF. However, following corrective surgery pulmonary valve replacement (PVR) might be required for residual pulmonary regurgitation in order to avoid irreversible right ventricular remodeling. Otherwise, residual uncorrected pulmonary regurgitation may lead to right ventricular dilatation, impaired biventricular function, ventricular arrhythmias and limited exercise capacity. We report the first case of Freedom Solo stentless valve (Sorin Group, Saluggia, Italy) implantation in the pulmonary position in an adolescent with severe pulmonary insufficiency 12 years after the repair of TOF. Pericardial stentless valves may be an alternative choice for pulmonary valve replacement to improve right ventricular contractile recovery and remodeling after PVR and may have impact on long-term survival.     

Pulmonary valve preservation in Tetralogy of Fallot with a mildly hypoplastic annulus-should we do it?

Background  

The decision to preserve the pulmonary valve during intracardiac repair of Tetralogy of Fallot [TOF] is traditionally based on the intra-operative measurement of pulmonary annulus by a Hegar dilator as per Rowlatt’s table. We sought to evaluate if there can be flexibility in not using a transannular patch repair in Indian population with mildly hypoplastic pulmonary annulus.     

Anesthetic management for an infant with tetralogy of fallot with absent pulmonary valve

Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is a rare disease, and characterized by massive dilatation of pulmonary artery. Bronchial compression by the aneurysm-like dilated pulmonary artery sometimes results in severe respiratory compromise. Surgery including closure of VSD and right ventricular outflow construction is often needed in infancy. But, even after the surgical correction, patients often suffer from right ventricular failure due to right ventriculotomy and poor development of pulmonary capillaries. Therefore, preventing pulmonary hypertension is important during the perioperative period. We report an infant with TOF/APV. This 3 month-old boy developed respiratory distress suddenly. After the induction of anesthesia with sevoflurane, fentanyl, and vecuronium, continuous infusion of alprostadil and olprinone was started. Cardiopulmonary bypass (CPB) was instituted smoothly. VSD was closed and right ventricular outflow tract was reconstructed. Dilated pulmonary arteries were resected and plicated. Weaning from CPB was easy and postoperative course was uneventful with mild pulmonary hypertension. In the cases predisposed to right ventricular failure like TOF/APV, olprinone may be useful.     

Preservation of the pulmonary valve during intracardiac repair of tetralogy of Fallot.

From January 1981 to December 1990, intracardiac repair of tetralogy of Fallot in 148 pediatric patients, with one surgical death, was directed toward preservation of the native pulmonary valve. Using the accepted preoperative angiographic criterion for the pulmonary valve annulus area (PVA) of 1.8 cm2/m2, 85 patients were candidates for transannular right ventricular outflow patch (TAP). However, in 54 patients with a mean PVA of 1.5 cm2/m2 (range 1.06-1.76), the valve was preserved without using TAP because the morphological changes (cusp thickening and annular distensibility) seemed acceptable for preservation in view of its probable hemodynamic efficiency and growth potential. A morphological classification of pulmonary valve changes has evolved. Retrospectively, 24 (77%) of the 31 patients with TAP had moderate to severe cusp thickening and ring rigidity; this incidence was significantly higher (p < 0.001) than that in preserved patients (18 of 54 or 33%). The incidence of morphological changes increased with operative age; that is, 2 of 13 (15%) patients younger than 1 year versus 23 of 40 (58%) patients older than 4 years (p < 0.01). All 54 patients with preserved pulmonary valves were catheterized one month postoperatively. The intraoperative right to left ventricular systolic pressure ratio (RVP/LVP) decreased significantly (p < 0.005) in one month, from a mean of 0.79 (range 0.44-1.36) to 0.57 (range 0.36-0.97). The PVA increased from a mean of 1.5 to 1.9 cm2/m2 (range 1.20-2.65), and the rate of its increase was significantly larger (p < 0.005) as operative age decreased. Pulmonary valve regurgitation of greater than mild degree occurred in 8 of 54 (15%) patients with the valve preserved.(ABSTRACT TRUNCATED AT 250 WORDS)     

Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries

Patients with tetralogy of Fallot (TOF), pulmonary atresia (PA), and major aortopulmonary collateral artery (MAPCA) usually need some staged surgical procedures. There is no clear consensus to the initial procedure and also the most proper initial procedure is different in each case. We report here a case of 6-year-old girl with TOF, PA, and MAPCA. We performed right ventricular outflow tract reconstruction (RVOTR) for the initial procedure because her pulmonary artery was extremely diminutive. Her pulmonary artery got good growth after the palliative surgery. Five years later she underwent complete repair after two other surgeries and 1 interventional embolization. RVOTR is a useful procedure for pulmonary artery growth, but it should be well considered that what size of RVOTR is needed in each case.     

Anesthesia for high‐risk procedures in the catheterization laboratory

Recent advances in catheterization and imaging technology allow for more complex procedures to be performed in the catheterization laboratory. A number of lesions are now amenable to a percutaneous procedure, eliminating or at least postponing the need for a surgical intervention. Due to the increase in the complexity of the procedures performed, the involvement of anesthesiologists and their close collaboration with the interventional cardiologists have increased. It is important to understand the physiology and pathophysiology of the patients and to anticipate the plans and the potential complications in order to manage them. We are witnessing a rise in the number of complex interventions in newborns and infants, such as balloon valvotomy (critical aortic stenosis, pulmonary stenosis), radio frequency perforation (of pulmonary atresia and intact ventricular septum), right ventricular outflow tract stenting (in Tetralogy of Fallot), ductal stenting (in some ductus‐dependent pulmonary circulation), and combined with a surgical procedure (hybrid procedure for hypoplastic left heart syndrome). Multiple registries have been created in order to understand and improve outcomes of patients with congenital heart disease undergoing catheterization procedures and to develop performance and quality metrics, from which data regarding anesthetic‐related risks can be extrapolated. Experienced personnel and a multidisciplinary team approach with direct communication among the team members is a must to ensure anticipation and management of critical events when they occur.     

Successful management of early aortic root abscess with aortic homograft implantation in children after Ross operation

The Ross operation is the best surgical procedure for aortic valve replacement in children of all age groups. A 3.5 years old boy developed early autograft endocarditis (9 days) following a straightforward Ross operation. Due to progressive neo-aortic valve destruction and aortic root abscess extending to the mitral annulus and valve, the pulmonary autograft had to be removed. A cryopreserved aortic homograft with its attached mitral valve leaflet was used to reconstruct the left ventricular outflow tract and repair the native mitral valve defect.     

Tetralogy of Fallot: Yesterday and Today

Tetralogy of Fallot (TOF) is a cyanotic congenital cardiac defect that was first described by Stenson in 1672 and later named for Fallot, who in 1888 described it as a single pathological process responsible for (1) pulmonary outflow tract obstruction, (2) ventricular septal defect (VSD), (3) overriding aortic root, and (4) right ventricular hypertrophy. The surgical history of TOF began with the development of the systemic to pulmonary artery shunt (BT shunt) by Blalock, Taussig, and Thomas in 1944. Ten years later complete repair of TOF was performed by Lillehei using cross-circulation and by Kirklin with a primitive cardiopulmonary bypass circuit. Notable contributions by several other surgeons including Bahnson, Ebert, Malm, Trusler, Barratt-Boyes, and Castaneda would lead us into the modern era of surgery. Today, complete repair of TOF is performed before six months of age with low mortality (<2%). In select cases a modified version of the BT shunt is still performed as the initial procedure. Long-term survival rates are excellent (85%–90%). Adult survivors with TOF are an ever-increasing population and may require reintervention, surgically or catheter based. Promising future innovations include percutaneous pulmonary valve replacement, tissue-engineered autologous valves and conduits, and genetic manipulation. This article presents a review of TOF, including the history of surgical treatment, present-day approaches, and long-term outcomes.     

Congenital aortic stenosis

Recent advances in and controversies concerning the management of children with congenital valvular aortic stenosis are discussed. In neonates with critical aortic stenosis, improved survival has recently been reported after surgical open valvotomy and balloon valvuloplasty, although it is difficult at this point to compare the results of the two procedures and determine their differential indications. Good results have also been achieved after extended aortic valvuloplasty for recurrent aortic stenosis and/or insufficiency, but the length of follow-up in these patients is still short. The technique first reported in 1991 for bilateral enlargement fo a small annulus permits the insertion of an aortic valve 3-4 sizes larger than the native annulus. It entails no risk of distorting the mitral valve, damaging the conduction system or important branches of the coronary arteries, or resulting in left ventricular dysfunction. The Ross procedure is now widely applied in the West, with reports of early mortality rates of less than 5% and event-free survival rates of 80-90% during follow-up of 4-8 years. Longer follow-up and continued careful evaluation are required to resolve the issue of possible dilatation and subsequent neoaortic valve dysfunction and pulmonary stenosis due to allograft degeneration after pulmonary autograft root replacement in children.     

Surgical treatment of right ventricular outflow tract obstruction after Tetralogy of Fallot {S,D,I} repair

Situs solitus of viscera and atria, D-looped ventricle with atrioventricular concordance, and inverted normally related great arteries with ventriculoarterial concordance characterize the segmental anatomy of an extremely rare {S,D,I} variant of Tetralogy of Fallot. The main pulmonary artery is rightward of the aorta and the right coronary artery crosses over the right ventricular outflow tract from left to right to reach the right atrioventricular groove. Our patient had undergone a neonatal repair due to persistent cyanosis with the implantation of a pericardial infundibular patch after the diagnosis of Tetralogy of Fallot {S,D,I}. He presented with an annular right ventricular outflow tract obstruction at 19 months of age. We describe the surgical approach employing a transannular patch implantation for the relief of the annular right ventricular outflow tract obstruction.     

法洛四联症根治手术长期疗效观察

法洛四联症是最常见的紫绀型先天性心脏病,其解剖学基础是心室间交通,主动脉骑跨,肺动脉瓣下狭窄,以及由此导致的右心室肥厚。尽管法洛四联症根治术后患者长期生存率很高,但晚期生存患者需要面对严重并发症。进行性肺动脉反流导致的右心衰竭和心律失常是其晚期主要并发症和死亡原因。为此,我们对晚期并发症的发生和治疗的进展进行了综述,强调长期随访的重要性。     

The results of probe technique for transatrial repair of tetralogy of Fallot

OBJECTIVE: Total correction of classic tetralogy of Fallot (TOF) by transatrial approach has become a standard procedure with a principal theoretical aim of minimizing structural damage to the pulmonary pump. The most critical point in transatrial repair of TOF is infundibular dissection. Right atrial approach provides better surgical exposure for parietal extension of the infundibular septum when compared to a right ventricular approach. However, it is not always easy to determine the localization and amount of muscle bundles to be resected and this surgical maneuver requires experience. METHODS: Nineteen patients were reviewed who had repair of isolated TOF by this technique from 1993 to 2001. The mean age of patients was 5 +/- 2 years. Transatrial-transpulmonary approach was performed for all patients. To make the infundibular muscle-bundle resection easier and to determine the localization and amount of muscle bundle to be resected, we placed a Hegar dilator into the right ventricle through pulmonary arteriotomy. The muscle bundles between the dilator and the anterior leaflet annulus of the tricuspid valve were totally excised until the intraventricular part of the dilator and pulmonary annulus became completely visible. The area between the Hegar dilator and the margins of the ventricular septal defect (VSD) was left untouched. None of the patients had transannular patch. Tricuspid valve detachment in order to improve the exposure was done in 11 patients. All patients were followed up in our clinic at regular six-month intervals by echocardiography. RESULTS: There was no early or late mortality nor reoperation for residual VSD or residual right ventricle (RV) outflow obstruction. All patients were in NYHA class I. RV on the echocardiography was spared late dilatation and had a good late functional status. Eighteen patients had no or mild pulmonary regurgitation. One patient who had undergone tricuspid anterior leaflet detachment showed mild tricuspid insufficiency. CONCLUSIONS: On the basis of hemodynamic outcomes, this procedure for elective repair of TOF in selected cases gives excellent early and mid-term results.     

The potential impact of percutaneous pulmonary valve stent implantation on right ventricular outflow tract re-intervention.

OBJECTIVE: To assess the impact of a percutaneous technique for pulmonary valve implantation on the conventional surgical valve/conduit approach to right ventricular outflow tract re-intervention. METHODS: We have retrospectively reviewed our results following surgical or percutaneous re-intervention to the right ventricular outflow tract in both paediatric and adult groups. Between November 1998 and March 2004, 94 patients underwent surgical re-intervention to the right ventricular outflow tract. Percutaneous pulmonary valve implantation was introduced in October 2002 and 35 procedures were performed to March 2004. The median age was 26 years (6-65 years) in the surgical group and 16 years (9-39 years) in the percutaneous group. Tetralogy of Fallot was the commonest original diagnosis (64.9 and 62.9%, respectively). The predominant indication for re-intervention in the surgical group was pulmonary regurgitation (64.9%) compared to the percutaneous group in which it was homograft/conduit stenosis or a mixed lesion (68.6%). RESULTS: There has been one (1.1%) early death reported in the surgical series and none in the percutaneous group. In the surgical group 9 (9.6%) experienced a procedural complication whilst 3 (8.5%) of those undergoing a percutaneous valve experienced a significant procedural event necessitating urgent surgery. Important early morbidity was 8 (8.5%) in the surgical group and 2 (5.7%) in the percutaneous group. Freedom from re-operation at 1 year was 100% in the surgical group and 86.1% in the percutaneous group due to late restenosis. Median hospital stay in the surgical group was 7 (4-114) days and 2 (2-22) days in the percutaneous group. CONCLUSIONS: Preliminary data suggests that percutaneous pulmonary valve implantation provides a promising additional and complementary approach to a successful surgical programme. Both approaches are safe with acceptable levels of morbidity and low mortality. With current technology the aneurysmal outflow tract remains a problem for the percutaneous approach. Follow-up remains too short, at present, to prove longevity of the percutaneous conduit.     

Indications for and clinical outcome of the Ross procedure: a review

The Ross procedure has been used increasingly to treat aortic valve disease in children and young adults. The primary indication for the Ross procedure is to provide a permanent valve replacement in children with congenital aortic stenosis. More recently, it has been extended to young adults with a bicuspid aortic valve and small aortic annulus, especially women wishing to have children. Other possible indications include complex left ventricular outflow obstructive disease, native or prosthetic valve endocarditis, and adult aortic insufficiency with a dilated aortic annulus. Conversely, Marfan syndrome is considered to an absolute contraindication, and this procedure should be used with caution in patients with rheumatic valve disease and a dysplastic dilated aortic root because of the higher associated incidence of autograft dysfunction. The technique of total aortic root replacement has become the preferred method of autograft implantation, because it carries the lowest risk of pulmonary autograft failure. In patients with marked graft-host size mismatch, either concomitant aortic annulus reduction and fixation or aortic annulus enlargement (i.e., the Ross-Konno procedure) should be performed. The Ross Procedure International Registry data document that in the modern era (post-1986) the early and late mortality rate is 2.5% and 1%, respectively. Excellent long-term results have been reported, and the benefits of this procedure include optimal hemodynamics, low risk of endocarditis, resistance to infection in patients with active endocarditis, and nonthrombogeneicity and therefore few anticoagulation-related complications. The Ross procedure can be performed with acceptable early and mid-term mortality and excellent autograft durability. Further long-term follow-up will confirm the role of this procedure in patients with various types of aortic valve disease.     

Fan-shaped expanded polytetrafluoroethylene valve in the pulmonary position

BACKGROUND: Residual pulmonary insufficiency remains a problem after transannular repair in pediatric patients. We have developed fan-shaped monocuspid and bicuspid valves made of expanded polytetrafluoroethylene (ePTFE) membrane for repair of right ventricular outflow tract obstruction in patients with tetralogy of Fallot. METHODS: We studied 20 patients (mean age, 39.6 months) with tetralogy of Fallot. The right ventricular outflow tract was reconstructed with use of a short, wide transannular patch and a fan-shaped valve made of 0.1-mm-thick expanded ePTFE membrane (PRECLUDE Pericardial Membrane, W.L. Gore & Associates, Inc, Flagstaff, AZ). The width of the valve and the outflow patch were constructed so that the neopulmonary annulus was approximately 130% the normal size. RESULTS: A monocuspid valve was used in 17 patients and a bicuspid valve in 3. The mean follow-up time was 32.3 months (range, 14.7 to 56.7 months). Right ventricular function was well maintained in all patients. Valvular motion remained competent in 13 patients (65%). In the remaining 7 (35%), the valve became fixed in the open position. Trivial and mild pulmonary insufficiency was detected in 10 patients with a competent valve and 7 with an incompetent valve. No pressure gradient across the neopulmonary annulus or calcification of the expanded ePTFE membrane was detected in any patient. CONCLUSIONS: The fan-shaped expanded ePTFE valve is a useful substitute with adequate function in transannular right ventricular outflow reconstruction.