Viktor Hraška

Functional outcome of anatomic correction of corrected transposition of the great arteries

OBJECTIVEnAnatomic correction of corrected transposition of the great arteries, utilizing the morphologic left ventricle as a systemic pumping chamber, is considered the preferred method. The purpose of the study was to analyze the intermediate functional outcome following anatomical correction.nnnMETHODSnBetween 1997 and 6/2010, 23 patients with corrected transposition of the great arteries and associated lesions underwent anatomical correction. Seventeen (74%) and six patients (26%) had situs solitus {S,L,L} and situs invs {I,D,D}, respectively. Fifteen patients (65%) had undergone 18 palliations before the corrective operation. The median age at palliation was 0.23 years, with a range of 0.016-8.4 years. A corrective, modified Senning-arterial switch procedure was performed in nine patients, 13 patients underwent a modified Senning-Rastelli procedure, and in one patient a combination of modified Senning and aortic translocation (Bex/Nikaidoh) was used. The median age at the corrective operation was 2 years (from 0.3 to 15.7 years).nnnRESULTSnThere was no mortality or heart transplant within the mean follow-up of 3.4 years. Freedom from reintervention was 77% at 5 years. There were no signs of obstruction of the systemic and pulmonary venous tunnels. The function of both ventricles was normal in all patients, even in the four patients who required retraining of the left ventricle. Mild aortic regurgitation was noticed in three patients. Preoperatively detected significant tricuspid regurgitation either disappeared or became trivial after the operation in all the six patients. All patients except two are in sinus rhythm; one patient is pacemaker-dependent preoperatively and one is pacemaker-dependent postoperatively. There were no clinically apparent neurological problems. All patients, but one, are in the New York Heart Association (NYHA) class I.nnnCONCLUSIONSnAnatomic correction of corrected transposition of the great arteries can be performed in selected patients without mortality and with acceptable morbidity. The mid-term functional outcome is excellent, resulting in normal ventricular function, even in retrained left ventricles, and minimal incidence of complete heart block. The long-term function of the aortic valve, intraventricular tunnels, conduits, and ventricles requires close surveillance.

Pulmonary artery sling with tracheal stenosis

Pulmonary artery sling (PAS) is a rare congenital heart disease in which the left pulmonary artery (LPA) originates from the right pulmonary artery (RPA) and encircles the distal trachea and right mainstem bronchus as it courses between the trachea and esophagus. Typically, patients with PAS have some respiratory symptoms, either due to external tracheal compression that can be corrected by relief of the sling mechanism, or due to severe diffuse tracheal stenosis with complete rings (ring-sling complex). The diagnosis of PAS is optimally made by echocardiography, while bronchoscopy is the key to the assessment of tracheal stenosis. Diagnosis is indication for surgery. Repair using a strategy of median sternotomy, cardiopulmonary bypass, division of the LPA and reimplantation into the main pulmonary artery (MPA), and simultaneous tracheal repair takes preference. Tracheal repair should be considered only in clinically symptomatic patients. The techniques of free tracheal autograft plasty or slide tracheoplasty offer promising results, and the choice of tracheal reconstruction should be guided by the clinical experience of the surgeon. Coexisting intracardiac pathologies are repaired at the same time. Postoperative care requires close multidisciplinary effort to achieve the best long-term result.

The modified Senning procedure as an integral part of an anatomical correction of congenitally corrected transposition of the great arteries.

In the current era of anatomical correction of complete transposition of the great arteries, the Senning operation is reserved only for the atrial switch part of anatomical corrections of congenitally corrected transposition of the great arteries. These rare operations are performed in only a few centers all over the world; the majority of practicing cardiac surgeons therefore have limited experience with the Senning operation. The proposed modified Senning procedure might simplify the original concept. Once the technical aspect of the procedure is accomplished, the risk of systemic and pulmonary baffle obstructions is minimal, even in situs solitus with dextrocardia or situs inversus with levocardia. Furthermore, this technique has the potential to provide adequate capacity of the pulmonary venous atrium, to preserve optimal geometry of the mitral valve, to minimize damage of sinus node and to make the coronary sinus accessible for electrophysiological studies or intervention by keeping the coronary sinus in the systemic venous baffle. The modified technique is simple, highly reproducible and applicable, regardless of the situs and position of the apex of the heart.

Ross-Konno operation in children

The Ross-Konno procedure is an excellent technique for the treatment of complex multilevel left ventricular outflow tract obstruction with severe annular hypoplasia and a dysplastic aortic valve. The operation can be performed earlier in life, thus avoiding repeated surgical reinterventions, which may provide only short-term palliation and potentially exacerbate ventricular function. The Ross-Konno procedure increases our therapeutic choices for neonates or infants with critical aortic stenosis, who show unacceptable results following open valvotomy or balloon valvotomy. The pulmonary autograft demonstrates durability without the likelihood of developing aortic stenosis or progressive dilatation and a low incidence of developing aortic insufficiency. Despite the technically demanding nature of the operation, the Ross-Konno procedure is the method of choice for the multilevel type of left ventricle outflow tract obstruction, especially in newborns and infants.

Surgery for tetralogy of Fallot – absent pulmonary valve syndrome. Technique of anterior translocation of the pulmonary artery

Patients with absent pulmonary valve syndrome (APVS) and tetralogy of Fallot represent a particular managements challenge. The distinctive feature of APVS is the airway obstruction caused by tracheobronchial compression that results from massive dilatation of the main pulmonary artery (PA) and its first- and second-order branches. Consequential tracheomalacia and bronchomalacia determine the timing and severity of respiratory compromise, as well as the morbidity and mortality of these patients. Although several techniques have been proposed for decompression of the airways, the method of choice is still controversial. Translocation of the PA anterior to the aorta and away from the airways has the potential to reduce or eliminate bronchial compression. Overall, the intermediate-term functional outcomes using this technique have been encouraging, with zero mortality even in the youngest age group of patients and the disappearance of respiratory symptoms in the majority of them. Nevertheless, failure of the treatment could be expected in symptomatic patients in whom the pathology of the airways extends beyond the proximal PAs.

Neurodevelopmental Outcome in Children With Single Ventricle After Total Cavopulmonary Connection

BACKGROUNDnThe purpose of the present study was to assess a broad range of neuropsychological outcome variables in children with functionally single ventricle hearts after a total cavopulmonary connection and to examine potential risk factors for impaired neurodevelopment.nnnPATIENTS & METHODnA total of 104 patients aged 2 to 20 years underwent follow-up standardized psychological testing, including measures of intelligence, motor function, visuospatial abilities, behavioural outcome and health-related quality of life.nnnRESULTSnWith a mean fluid intelligence score of 93.0 and a mean crystallized intelligence score of 92.3, patients scored significantly lower on tests of intelligence compared to the general population (p<0.001). Reduced motor function was found in 34% of the patients (p<0.001), impaired visuospatial abilities in 51%; parents reported significantly more behavioural problems (p<0.001). There was no difference in the self-reported quality of life compared to the general population. Risk factors for reduced fluid intelligence were deep hypothermic circulatory arrest times (p=0.03) and complications causing suboptimal brain perfusion prior to the total cavopulmonary connection (e.u2009g. seizures; p=0.04).nnnCONCLUSIONSnPatients with functionally single ventricle hearts palliated with a total cavopulmonary connection are at an increased risk of neurodevelopmental delays and behavioural disorders. Nevertheless, they adapt well in terms of quality of life. Early diagnostics and interventions are necessary when developmental delays are suspected.

Congenitally corrected transposition of the great arteries

MD Gerd Geffert Practice of Cardiology 25813 Husum, Germany A 58 year-old woman was admitted with progressive shortness of breath. Previously, she had been diagnosed with idiopathic dilated cardiomyopathy, with moderate mitral regurgitation and suspected apical left ventricular thrombus. Coronary artery disease was angiographically excluded at that time. The echocardiography and cardiac MRI done at admission revealed profound dilation of the systemic, left-sided anatomical right ventricle, with prominent trabecularization of the apex (Fig. 1 a and 1 b, Movie 1). The systolic function of the systemic ventricle was depressed with moderate regurgitation of the left-sided tricuspid valve (Movie 2). The aorta was located anteriorly and to the left (Fig. 2, Movie 3). The angio-CT documented the right coronary artery arising from the left posterior sinus and the left coronary artery coming from the right coronary sinus (Fig. 3).

Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome is characterized by severe underdevelopment of the structures in the left heart-aorta complex, including the left ventricular cavity and the aortic arch. This chapter elaborates:

Tetralogy of Fallot

Evidence suggests that early correction minimizes secondary damage to the heart or other organ systems due to chronic hypoxia, promotes pulmonary artery growth, and alleviates the stimulus for continuous right ventricular hypertrophy, thus preserving the mechanical and electrical stability of the heart. In the majority of centers, therefore, primary repair is electively performed before 6 months of age. The strategy of primary repair provides excellent outcomes, with mortality approaching zero, and acceptable morbidity. Avoidance of a shunt also has economical and psychosocial advantages. A combination of transatrial and transpulmonary approach is the preferred method. An effort is made to preserve the pulmonary valve, thus potentially limiting the negative impact of pulmonary regurgitation on right ventricular function. The need for a transannular patch is determined by the hypoplastic pulmonary artery annulus, and it is not eliminated by the shunt procedure. If a transannular approach is unavoidable, excision and patching should be minimal to prevent the long-term adverse sequelae associated with right ventriculotomy, particularly in the presence of pulmonary insufficiency. Depending on the institutional experience and policy, the staged approach remains a reasonable option.

Corrected Transposition of the Great Arteries

Congenitally corrected transposition is a rare condition, characterized by atrio-ventricular and ventricular–arterial discordance. The clinical presentation and indication for surgery generally depends on the associated cardiac lesions such as ventricular septal defect, obstruction of the outflow tract from the morphologically left ventricle, abnormalities of the morphologically tricuspid valve, and problems with the conduction system.