Aleksandra Morka

Patient-tailored aortic root repair in adult marfanoid patients: Surgical considerations and outcomes

Objective The aim of the study was to evaluate the operative and functional results after individual, patient‐tailored aortic root repair in marfanoid patients. Methods Among 518 patients who underwent operation between 2002 and January 2016, using patient‐tailored aortic root repair with isolated sinus replacement, 42 patients fulfilled the original Ghent criteria. None/trivial, mild, moderate, and severe insufficiency grades were present in 5, 16, 10, and 11 patients, respectively. Results The repair was adjusted to the existing aortic annulus diameter. Replacement of 1, 2, or 3 sinuses of Valsalva was performed in 1, 14, and 27 patients, respectively. Concomitant cusp repair was performed in 17 patients (40.5%), and 10 patients (23.8%) underwent arch repair (total in 3). All patients survived surgery, and the follow‐up (mean, 6.1 ± 3.1; range, 0.8‐14.2 years) was 100% complete. No patient had a change in the form or size of the aortic neo‐root, especially the size of aortic annulus and sinotubular junction during the follow‐up time. No and trivial/mild insufficiency were present in 22 and 18 patients, respectively, and 2 patients with recurrent aortic insufficiency caused by cusp pathology underwent aortic valve replacement 43 and 66 months after the primary surgery, respectively. Thus, the estimated survival free from aortic valve/root reoperation for any reason at 5 and 8 years was 96.8% ± 3.2% and 91.4% ± 6.0%, respectively. Conclusions Patient‐tailored root repair using isolated sinus replacement is an effective and durable method of valve‐sparing repair in select marfanoid patients with a satisfactory quality of aortic cusps, which seems to be decisive for long‐term valve function.

Left Ventricular Diastolic Dysfunction Assessed by Conventional Echocardiography and Spectral Tissue Doppler Imaging in Adolescents With Arterial Hypertension

AbstractCompared to conventional echocardiography, spectral tissue Doppler imaging (s-TDI) allows more precise evaluation of diastolic cardiac function. The purpose of this study was to conduct s-TDI to analyze the slow movement of the left ventricular (LV) myocardium in adolescents with systemic arterial hypertension (HT) and to determine whether patients with HT suffer from LV diastolic dysfunction. The study group comprised 69 consecutive patients (48 boys and 21 girls aged 14–17 years [mean, 15.5 ± 1.1 years]) with primary HT, and the control group comprised 48 healthy participants (24 boys and 24 girls aged 14–17 years [mean, 15.8 ± 1.3 years]). Physical examinations, 24-hour arterial blood pressure monitoring, conventional 2-dimensional and Doppler echocardiography, and s-TDIs were performed. Analysis revealed that study group participants were significantly heavier and had greater LV mass indices than controls (P < 0.001). There were no differences between the velocities of E waves (peak early filling of mitral inflow), but the deceleration times of the mitral E waves were significantly shorter whereas the A waves survived longer in the study group than in the control group. The velocities of A waves (peak late filling of mitral inflow) were elevated (P = 0.041), and the E/A wave pattern (E/A = 1.8 ± 0.4) was normal. These results suggest pseudonormalization, a type of LV diastolic dysfunction in adolescents with HT.In the study group, when the sample volume was positioned at the septal or lateral insertion site of the mitral leaflet, the e′ wave velocity was significantly depressed whereas the a′ wave velocity was elevated, compared to those of the control group (P < 0.001).The e′/a′ ratios from the septal and lateral insertion sites were lower, whereas the E/e′ ratio from the septal insertion site was significantly higher in the study group, similar to that seen in atrial reversal velocity (P < 0.001).These findings indicate that using sTDI to find and measure diastolic LV failure is valuable when the probe is placed at the septal and lateral mitral valve annuli during examination.Changes in the myocardium appear similar to those seen in adults.

Validation of Standard and New Criteria for the Differential Diagnosis of Narrow QRS Tachycardia in Children and Adolescents.

AbstractTo establish an appropriate treatment strategy and determine if ablation is indicated for patients with narrow QRS complex supraventricular tachycardia (SVT), analysis of a standard 12-lead electrocardiogram (ECG) is required, which can differentiate between the 2 most common mechanisms underlying SVT: atrioventricular nodal reentry tachycardia (AVNRT) and orthodromic atrioventricular reentry tachycardia (OAVRT). Recently, new, highly accurate electrocardiographic criteria for the differential diagnosis of SVT in adults were proposed; however, those criteria have not yet been validated in a pediatric population.All ECGs were recorded during invasive electrophysiology study of pediatric patients (n = 212; age: 13.2 ± 3.5, range: 1–18; girls: 48%). We assessed the diagnostic value of the 2 new and 7 standard criteria for differentiating AVNRT from OAVRT in a pediatric population.Two of the standard criteria were found significantly more often in ECGs from the OAVRT group than from the AVNRT group (retrograde P waves [63% vs 11%, P < 0.001] and ST-segment depression in the II, III, aVF, V1–V6 leads [42% vs 27%; P < 0.05]), whereas 1 standard criterion was found significantly more often in ECGs from the AVNRT group than from the OAVRT group (pseudo r′ wave in V1 lead [39% vs 10%, P < 0.001]). The remaining 6 criteria did not reach statistical significance for differentiating SVT, and the accuracy of prediction did not exceed 70%. Based on these results, a multivariable decision rule to evaluate differential diagnosis of SVT was performed.These results indicate that both the standard and new electrocardiographic criteria for discriminating between AVNRT and OAVRT have lower diagnostic values in children and adolescents than in adults. A decision model based on 5 simple clinical and ECG parameters may predict a final diagnosis with better accuracy.

Hypoplastic left heart syndrome with anomalous circumflex artery arising from left pulmonary artery. A description of combined surgical and interventional cardiac treatment

Corresponding author: Magdalena Czerżyńska MSc, Department of Pediatric Cardiac Surgery, Jagiellonian University Medical College, 265 Wielicka St, 30-663 Krakow, Poland, phone/fax: +48 501 540 689, e-mail: mmagdalenacz@gmail.com Received: 4.01.2018, accepted: 23.04.2018. Hypoplastic left heart syndrome with anomalous circumflex artery arising from left pulmonary artery. A description of combined surgical and interventional cardiac treatment

Perventricular Implantation of Melody Valve in Child With Pulmonary Hypertension After a Potts Shunt

The Potts operation is a surgical option for the treatment of children with suprasystemic pulmonary arterial hypertension (PAH). Its positive hemodynamic effect may be obliterated by pulmonary valvar insufficiency. We describe a case in which the Potts shunt was followed by perventricular Melody valve implantation in the pulmonary position in a 12-kg child with a previously repaired truncus arteriosus communis (TAC) in whom PAH developed. The combined approach using both procedures significantly improved functional capacity and led to spectacular reverse remodeling of the right ventricle seen at 2-year follow-up. Elimination of significant pulmonary insufficiency is crucial in achieving the hemodynamic benefits of a Potts shunt in patients with pulmonary hypertension.

Cardiac fibroma in a neonate

Cardiac tumours in children are rare; they often require immediate surgical intervention. Assertive, even aggressive, operations constitute the method of choice in the majority of cases. The author describes a female neonate (pregnancy 3, delivery 2) born by C-section at 38 weeks with a giant, extremely rare right ventricle (RV) tumour and impending cardiac tamponade. The 32-year-old mother presented with hypothyroidism and obesity. Prenatal ultrasonography at 24 weeks of gestation showed no abnormalities or structural cardiac and vascular lesions, possibly indicating an extremely dynamic tumour growth in the third trimester. On admission, physical examination showed significant dyspnoea and heart rate of 170/min. Immediately performed echocardiography demonstrated a giant tumour of the RV free wall, 20 × 30 mm in diameter, 6 cm2 in surface area, which was non-encapsulated, showing no borderline between the ventricular muscle and tumour margins. The tumour mass was non-uniform, with hyper and hypoechogenic foci, significantly narrowing the RV lumen in the inflow tract, restricting the movement of the anterior and posterior tricuspid valve (TV) leaflet. TV — 5 mm opening, I/II° regurgitation with maximum RV/RA systolic gradient of approximately 22 mm Hg; left ventricle (LV) — 4 mL in volume, with normal systolic function (CiAo 2.4 L/min/m2); mitral valve — 0.97 cm; tricuspid competent aortic valve with 0.85 cm annulus; normal ostium and initial course of the coronary arteries. Pulmonary artery trunk and branches normal, no patent ductus arteriosus. Left aortic arch, normal aortic isthmus (Fig. 1). In view of the girl’s state, angio-computed tomography was not performed. Following pharmacological stabilisation, she was prepared for surgery. Under cardiopulmonary bypass and deep hypothermia, subtotal tumour resection was performed involving the masses situated on the anterior cardiac wall and blocking the inflow to the TV. The post-resection margins on the anterior heart surface were approximated with continuous sutures. The resection was performed within the normal tissue limits, exercising extreme caution around the coronary circulation vessels. Tumour consistency resembled hard, cheese-like, poorly vascularised masses with numerous calcifications (Figs. 2, 3). The material was referred to histopathology, which confirmed the suspicion of fibroma. Having warmed the patient, aided by catecholamines and milrinone, cardiopulmonary by-pass was discontinued. Initially, her postoperative status was severe, with low arterial pressure requiring volaemia supplementation, levosimendan, and increased amine administration. Postoperative transthoracic echocardiography — TV diameter 6 mm, sub-TV tumour — 16 × 17 mm with non-uniform structure, restricting movement of the anterior TV cup, but showing significant inflow improvement as compared to preoperative results, decreased RV volume in the inflow tract, preserved RV trabecular outflow tract. Pulmonary valve diameter of approximately 9 mm (preserved blood outflow to the pulmonary trunk from the RV side); CiPV < 1 L/min/m2; CiAo approximately 3 L/min/m2; LV 1.6–1.7 cm; ejection fraction 70–75% (Fig. 4). In subsequent days, improvement of general status and echocardiographic picture was achieved; the latter showed regression of tumorous lesions that were non-resectable due to the risk of damaging important heart structures. She was extubated on day 10 postoperatively. Further postoperative course was uncomplicated. She is presently developing normally and is being followed-up by out-patient cardiology department. No recurrent fibroma has been seen, and tumour remnants are gradually disappearing. Assertive surgical intervention in such extremely dangerous cases is often the only correct management method. Figure 1. Transthoracic echocardiography right ventricular tumour

Comparison of the Degree of Exercise Tolerance in Children After Surgical Treatment of Complex Cardiac Defects, Assessed Using Ergospirometry and the Level of Brain Natriuretic Peptide.

AbstractChildren who underwent surgery for complex congenital heart defects present worse exercise capacity than their healthy peers. In adults and adolescents, heart failure is assessed on the basis of clinical symptoms using the New York Heart Association (NYHA) score, while in an infant Ross scale; heart failure can also be evaluated by other parameters. The purpose of this study was to compare the degree of exercise tolerance in children after surgery for complex heart defects, assessed by the ratio of maximum oxygen uptake (VO2max) and the brain natriuretic peptide (N-terminal fragment of the prohormone brain-type natriuretic peptide [NT-proBNP]) concentration.The study group consisted of 42 children, ages 9 to 17 years (mean 14.00 ± 2.72). Among them there were 22 children with tetralogy of Fallot (ToF) after total correction, 18 children with transposition of the great arteries (d-TGA) after the arterial switch operation, and 2 children with single ventricle (SV) after the Fontan operation. All but 1 child were in NYHA class I. The control group consisted of 20 healthy children. Outcomes of interest were the ratio of VO2max, determined during ergospirometry, and the level of NT-proBNP. The statistical analysis was performed and the groups were considered significantly different for P < 0.05.There was no statistically significant correlation between NT-proBNP and maximum oxygen uptake (VO2) kg−1 min−1 in the study group compared with the control group.The VO2max in the test group had a mean value less (34.6 ± 8.0) than controls (38.4 ± 7.7), and the differences were statistically significant (P = 0.041). In contrast, the average concentration of NT-proBNP in the study group was higher than controls (117.9 ± 74.3 vs 18.0 ± 24.5), and these differences were statistically significant (P < 0.001).After operations for complex heart defects (ToF, TGA, and SV), children have worse heart function parameters and exercise capacity than the healthy population. To control this, we recommend postoperative ergospirometry and determination of NT-proBNP concentrations.