Krisztina Kádár

Rapid recurrence of an inflammatory myofibroblastic tumor in the right ventricular outflow tract

We describe an unusual case of rapid recurrence of a previously excised inflammatory myofibroblastic tumor of the right ventricular outflow tract in a 5-month-old infant. The infant is asymptomatic 18 months after the second surgery. The very rare cardiac involvement, and the early relapse of the inflammatory pseudotumor, to the best of our knowledge, is a unique combination. The inflammatory myofibroblastic tumor, as known as a pseudotumor or plasma cell granuloma is an uncommon reactive lesion with unknown aetiology. It is found most commonly in the lung and a number of visceral organs, such as the spleen, liver, ileum, salivary glands, urinary bladder, larynx or brain or in the retroperitoneum and lymph nodes. To our knowledge only 9 cases have been published of such tumor arising within the heart.

Transposition of Great Arteries Is Associated With Increased Carotid Artery Stiffness

Transposition of great arteries is the consequence of abnormal aorticopulmonary septation. Animal embryonic data indicate that septation and elastogenesis are related events, but human and clinical data are not available. We tested the hypothesis that large artery elastic function was impaired in patients with transposition of great arteries. We studied 34 patients aged 9 to 19 years, 12±3 years after atrial switch operation; 14 patients aged 7 to 9 years, 8±1 years after arterial switch operation; and 108 healthy control subjects matched for age. Carotid artery diastolic diameter and pulsatile distension were determined by echo wall-tracking; carotid blood pressure was measured by tonometry. Systolic pressure was higher and diastolic pressure was lower in patients than in controls. Patients with atrial and arterial switch repair were compared with their respective controls by 2-factor ANOVA. For patients with atrial switch repair versus control, stiffness index &bgr; was 4.9±1.5 versus 3.1±1.0 (P<0.001); for patients witch arterial switch versus control, stiffness index &bgr; was 3.8±1.1 versus 2.1±0.6 (P<0.001). Similar differences were observed for carotid compliance, distensibility, and incremental elastic modulus as well. The interaction term was not significant for any of the elastic variables, indicating that carotid stiffening was a characteristic of the condition and not the consequence of different hemodynamics. Carotid artery is markedly stiffer in patients, suggesting that impaired elastogenesis may constitute part of the congenital abnormality. Since carotid artery stiffness has been established as an independent cardiovascular risk factor, this condition may have consequences in the clinical management of these patients.

Adaptation of baroreflex function to increased carotid artery stiffening in patients with transposition of great arteries

We have shown previously that TGA (transposition of great arteries) is associated with increased carotid artery stiffness. It has been established that stiffening of the barosensory vessel wall results in reduced baroreceptor activation and impaired BRS (baroreflex sensitivity). In the present study we tested the hypothesis that the increased carotid artery stiffness in TGA patients was associated with reduced cardiovagal BRS. We studied 32 TGA patients aged 9-19 years, 12+/-3 years after surgical repair and 32 age-matched healthy control subjects. Carotid artery diastolic diameter and pulsatile distension was determined by echo wall tracking; carotid blood pressure was measured by tonometry. BRS was measured using spontaneous techniques [BRS(seq) and LF(gain) (low-frequency transfer function gain)] and by the phenylephrine method (BRS(phe)). Carotid artery distensibility was markedly reduced in patients as compared with controls (5.6+/-1.9 x 10(-3) compared with 8.7+/-2.7 x 10(-3)/mmHg P<0.05, as determined using an unpaired Students t test), but BRS was not different in patients and controls (20.3+/-14.7 compared with 21.7+/-12.7 for BRS(seq); 13.1+/-9.2 compared with 10.6+/-4.5 for LF(gain); and 19.1+/-8.6 compared with 24.8+/-7.2 for BRS(phe) respectively). Carotid artery elastic function was markedly impaired in patients with TGA, but the increased stiffness of the barosensory vessel wall was not associated with reduced BRS. It appears that attenuation of baroreceptor stimulus due to arterial stiffening may be compensated by other, possibly neural, mechanisms when it exists as a congenital abnormality.

Impaired baroreflex function is related to reduced carotid artery elasticity in patients with tetralogy of Fallot

Sudden cardiac death (SCD) is a common late complication in patients with tetralogy of Fallot (ToF). Reduced baroreflex sensitivity (BRS) is an independent predictor of SCD and BRS reduction was reported in ToF. Relationship between BRS and carotid artery distensibility (DC) in healthy subjects was reported by us earlier. We also found that DC was reduced in ToF patients. In the present study we tested the hypothesis that reduced BRS is related to increased carotid artery stiffness. We studied 36 ToF patients (21±11 years) and 60 age-matched healthy control subjects. Intravenous phenylephrine-induced (BRSphe) and spontaneous (BRSseq) BRS indices were derived. DC calculation was based on echo wall-tracking and tonometry. BRS indices were reduced in patients compared with controls (BRSphe 16.8±10.2 vs. 27.3±9.2ms/mmHg; BRSseq 9.3±9.2 vs. 18.3±7.8ms/mmHg). DC was also lower in patients (5.1±1.8 vs. 6.3±2.610(-3)/mmHg). BRS correlated with DC across patients and controls (BRSphe r=0.75 vs. r=0.74; BRSseq r=0.44 vs. r=0.38). Multiple regression analysis indicated that BRS indices are determined independently by DC in ToF patients. We showed that reduced DC may contribute to impaired baroreflex function in ToF patients and could in part explain the elevated risk for SCD postoperatively. Therefore it would be an important future investigation to test carotid artery stiffness and analyze its predictive value for cardiac mortality in ToF. Preventive actions to impede carotid artery stiffening should receive more attention in the clinical management of ToF patients.

Aortic root abscess with aortico-mitral discontinuity after a ross operation in a child: reconstruction with an oversized homograft.

Abstract  A case of early neo‐aortic root abscess following a Ross operation in a 3½‐year‐old child is described. The infection destroyed neo‐aortic wall with two of the semilunar leaflets detached, and the abscess cavity extended across the intervalvular fibrous body into the left atrium and onto the native mitral valve creating partial “aortico‐mitral discontinuity.” Reconstruction was successful with an oversized cryopreserved homograft. (J Card Surg 2004;19:155‐157)

Redo procedures for correction of the aortic valve diseases in infants and children. The Bentall–Konno procedure

On an infant with critical valvular aortic stenosis balloon-valvoplasty, and 3 years later because of the aortic valve regurgitation Ross operation was performed. In the early postoperative period an aortic-root abscess occurred due to an infective endocarditis; the aortic root was corrected by homograft implantation. Due to a relatively small, calcified aortic valve, with aortic valve regurgitation grade III at the age of 12 years, a Bentall-Konno procedure was performed successfully. This is the first case when this complex surgical procedure was performed successfully on a child in Hungary.

Több-billentyűs redoműtét felnőtt betegen komplex pulmonalis atresiában

30-year-old adult with complex pulmonary atresia (previous surgical procedures: in infancy: exploration, at age of 10: ventricular septal defect closing, unifocalization, homograft implantation between right ventricular outflow tract and pulmonary artery) has biventricular dysfunction because of aortic valve regurgitation, ascending aortic aneurysm, and homograft insufficienty. Multivalve surgery: aortic valve plasty, pulmonary homograft changes for homograft and ascending aortic reconstruction by graft were carried out successfully. In Hungary this was the first case of this type of surgery. Management of special problems (follow-up, correct diagnostics (echocardiography, MR, CT), indication and necessity of reoperation, optimal age) in adult patients with complex congenital heart defects produces excellent early and late surgical results. Orv. Hetil., 2017, 158(14), 546-549.Absztrakt: A 30 eves, komplex pulmonalis atresias betegnel csecsemőkorban az unifokalizalas eldonthetősege miatt exploratio, 10 eves korban rekonstrukcios műtet (kamrai sovenyhiany zarasa, unifokalizacio, jobb kamra–arteria pulmonalis folytonossaganak kialakitasa homografttal) tortent. A mindket kamra csokkent műkodeset okozo aortabillentyű-elegtelenseg es ascendenstagulat, illetve homograftelegtelenseg miatt az aortabillentyű plasztikaja, a pulmonalis billentyű, illetve az aorta ascendens es arteria pulmonalis torzs csereje valt szuksegesse. Magyarorszagon betegunk az első, akinel ilyen redoműtet tortent. A műtet sikere igazolja, hogy megfelelő gondozas, diagnosztika (echokardiografia, MR, CT) mellett az időben tortent műtet jo korai es kesői eredmenyt biztosithat a komplex szivhibas, felnőttkoru betegek szamara. Orv. Hetil., 2017, 158(14), 546–549.

Aorta–bal kamra tunnel sikeres sebészi kezelése modern noninvazív képalkotó diagnosztika alapján

Aortico-left ventricular tunnel is a rare congenital cardiac defect, which bypasses the aortic valve via the paravalvar connection from the aorta to the left ventricle. The authors present the case of a 14-year-old boy with aortico-left ventricular tunnel in whom the aortic orifice arose from the right aortic sinus and was closed by a pericardial patch. The diagnosis was confirmed by combined two-dimensional and real time three-dimensional echocardiogram and magnetic resonance imaging. This is the first case, in which these complex diagnostic imaging methods have been used in the pre- and postoperative management of this defect. Optimally the new transthoratic three-dimensional echocardiography would be needed to define the anatomy and functional consequences of the aortico-left ventricular tunnel and in the postoperative follow-up.Aortico-left ventricular tunnel is a rare congenital cardiac defect, which bypasses the aortic valve via the paravalvar connection from the aorta to the left ventricle. The authors present the case of a 14-year-old boy with aortico-left ventricular tunnel in whom the aortic orifice arose from the right aortic sinus and was closed by a pericardial patch. The diagnosis was confirmed by combined two-dimensional and real time three-dimensional echocardiogram and magnetic resonance imaging. This is the first case, in which these complex diagnostic imaging methods have been used in the pre- and postoperative management of this defect. Optimally the new transthoratic three-dimensional echocardiography would be needed to define the anatomy and functional consequences of the aortico-left ventricular tunnel and in the postoperative follow-up.

The role of Ross procedure in the management of congenital heart defects

INTRODUCTION The surgical strategy to manage multilevel left ventricular outflow tract diseases is changing constantly, however, the Ross-procedure has remained a standard method for 45 years. AIM The aim of the study was to analyze early and mid-term results of Ross-procedure in congenital heart defects (single surgeons results). METHODS From 2001 until 2011 a total of 63 patients (age, 28 days-21 years; mean: 10 years, weight 3.4-96 kg; mean, 8.8 kg) underwent Ross (n = 40), Ross-Konno (n = 17) or Ross-Konno-mitral (n = 6) procedures. Indication for Ross procedure was aortic regurgitation in 15 patients (associated with ventricular septum defect in 8 patients) and a predominant aortic stenosis in 25 patients. 17 patients with severe left ventricular outflow tract obstruction underwent Ross-Konno procedure. 6 patients with concomitant mitral valve disease (Shone syndrome, 3 patients; complete atrioventricular septal defect, 3 patients) were reconstructed by Ross-Konno-mitral valve procedure. RESULTS Among Ross patients there were one early (cerebral complication) and one late death (homograft endocarditis) with a mean follow-up time of 7.4±1.8 years. Because of an early autograft endocarditis a 3-year-old boy underwent homograft implantation and was treated successfully with Bentall-procedure 9 years later. One patient with autograft regurgitation is waiting for reoperation. Among Ross-Konno patients there was no early or late death, and none of the patients underwent reoperation. In Ross-Konno-mitral patients there was one early death (28-day-old boy) and during a mean follow-up time of 2.5±1 years, and no reintervention or reoperation was needed in 5 patients. CONCLUSIONS The results indicate a good outcome of Ross-, Ross-Konno-, Ross-Konno-mitral procedures in patients with congenital heart defects when surgery is performed by a highly experienced heart surgeon. In newborns, infants and small children Ross- and Ross-Konno procedures are the only methods for managing left ventricular outflow tract diseases. Concomitant severe mitral disease adds a high level of technical complexity to the Ross-Konno/mitral procedure, but it should be balanced against alternative strategies (eg. single ventricle palliation or transplantation).

Correlations between B-type natriuretic peptide levels and nongeometric echocardiographic parameters in pediatric cardiomyopathies

INTRODUCTION B-type natriuretic peptide is a conventional cardiac biomarker in adult cardiology, however, it is not commonly used in pediatric cases. AIMS After introducing B-type natriuretic peptide measurements in pediatric patients for the evaluation of systemic right ventricular function, the authors wanted to evaluate the value of plasma B-type natriuretic peptide and compare the results with systolic and diastolic myocardial parameters obtained with conventional echocardiography and tissue Doppler imaging in children with cardiomyopathy. METHODS Between 2007 and 2010, 58 plasma B-type natriuretic peptide measurements were performed in 32 children (dilated cardiomyopathy in 20, hypertrophic cardiomyopathy in 10 and non-compacted cardiomyopathy in 2 cases). The age of the patients was 7.9±6.6 years (mean±SD). Plasma B-type natriuretic peptide was measured using an electrochemiluminescent assay within one day from echocardiographic evaluation. RESULTS As compared to normal values, children with the 3 types of cardiomyopathies showed significant differences in plasma B-type natriutretic peptide levels (dilated cardiomyopathy vs normal, p<0.001; hypertrophic cardiomyopathy vs. normal, p<0.01; non-compacted cardiomyopathy vs. normal, p<0.001). There was a significant negative correlation (r = -0,63; p<0,01) between B-type natriuretic peptide levels (range, 12-7002 ng/L; mean±SD, 1531±1750 ng/L) and linEF values (range, 4-50%; mean±SD, 22.5±13%). For B-type natriuteric peptide, a cut-off point of 1000 ng/L proved to differentiate significantly decreased linEF values (<17%). B-type natriuretic peptide levels significantly correlated with left ventricular end-diastolic diameter (r = 0.899; p<0.001), with left ventricular anular S wave parameters (r = 0.689; p<0.001) and with E/e ratio (r = 0.43; p<0.05). CONCLUSIONS B-type natriuretic peptide measurements are recommended in all types of cardiomyopathies.