Szymon Pawlak

Incomplete endothelialisation of an Amplatzer Septal Occluder device followed by meningitis and late acute bacterial endocarditis

A 19-year-old woman with atrial septal defect treated percutaneously with an Amplatzer Septal Occluder 24 months earlier, who presented with a history of bacterial meningitis, was admitted with a diagnosis of endocarditis. After 6 weeks of treatment with antibiotics, the incompletely endothelialised occluder was surgically removed. The present report illustrates the need for long-term follow-up of patients who have received nitinol wire mesh occluders.

Immediate and long-term outcomes of percutaneous transcatheter pulmonary valve implantation

BACKGROUND Transcutaneous pulmonary valve replacement (TPVR) has become an alternative to heart surgery for patients after previous right ventricular outflow tract (RVOT) or pulmonary artery (PA) surgical interventions. The objective was to present immediate and long-term outcomes of trans¬cutaneous pulmonary valve replacement. METHODS Between 06/2009 and 06/2016, 46 patients underwent TPVR. Initial diagnoses included tetralogy of Fallot, common arterial trunk, transposition of great arteries post Rastelli correction, left ventricle outflow obstruction after Ross operation, pulmonary atresia, and isolated dysplastic pulmonary valve stenosis. Thirty eight (78%) patients had previously implanted conduits in the pulmonary position, the rest had either RVOT patch reconstruction (n = 6; 13%) or biological valve implantation (n = 2; 4%). They presented primarily with pulmonary stenosis (n = 18; 39%) or regurgitation (n = 28; 60%). RESULTS All procedures were successful - 44 Melody and 2 Edwards-Sapien valves were implanted. Before each procedure exclusion of potential coronary compression and RVOT prestenting was performed. Significant RVOT systolic gradient reduction (from 35.3 ± 19.5 to 13.5 ± 7.1 mm Hg; p < 0.001) and decrease of right to left ventricle systolic pressure ratio from 0.58 ± 0.18 to mean 0.37 ± 0.1 (p < 0.001) was achieved. Also, in every patient PA-RVOT competence was restored, with minor in¬competence in only a few patients. Post procedure follow-up ranged from 2 to 86 (mean 35.2) months. Follow-up fluoroscopy or chest X-ray revealed 6 stent fractures (2 stent defragmentation - with only 1 significant valve stenosis). CONCLUSIONS Transcutaneous pulmonary valve replacement is a safe procedure with encouraging results, it also enables deferring surgical reintervention in the majority of patients.

Use of the AndraStent XL and XXL for the treatment of coarctation of the aorta in children and adults: immediate and midterm results.

AIMS The aim of this study is to present our experience with a new type of extra-large stent, namely the AndraStent XL/XXL. METHODS AND RESULTS AndraStents were implanted in 46 patients, 38 with native coarctation of the aorta (CoA) and eight with recurrent coarctation of the aorta following previous surgery (ReCoA). All patients had arterial hypertension in the upper limbs, absent or weak femoral pulses, and continuous flow in the abdominal aorta on Doppler examination. The median age of patients was 25 years (range from nine to 65). The stents (cobalt-chromium with semi-open cell design) were manually mounted on balloon catheters and delivered through 10 to 14 Fr Mullins sheaths using a conventional femoral approach. All procedures were successful (no stent migration, proper expansion, no aorta dissection/rupture, major bleeding or major vessel injury). The systolic gradient across the aorta decreased from 40.6 mmHg before the procedure to 11.6 mmHg after the procedure (p<0.001). The mean fluoroscopy time was 5.7 minutes. Imaging examinations were scheduled and performed between six and 12 months after the initial procedure in 40 patients (angiography or angio CT or both). Procedural outcome remained favourable during a mean 2.4 years of follow-up. Neither stent fracture nor dislocation was observed in any patient at follow-up. In two patients, small aneurysm formation was observed six and eight months after the procedure -they were treated successfully with subsequent covered stent implantation. Re-dilatation of the implanted stent was performed between four and 26 months in 14 patients. CONCLUSIONS Implantation of new large cobalt-chromium stents is a good therapeutic option for the treatment of native and recurrent CoA.

Aortic cusp extension valvuloplasty: repair with an extracellular patch.

Introduction The proportion of valve repair procedures is increasing in experienced centers. The aim of the study was to assess the clinical and echocardiographic outcomes after aortic valve reconstruction with a novel surgical technique. Material and methods The study group consisted of 30 patients (23 male and 7 female) at a mean age of 35 ± 14 years. In patients with aortic root aneurysm the reimplantation or Florida sleeve technique was used. A sub-commissural annuloplasty, plication of the free edge of the cusp, shaving, and commissurotomy were performed. At this stage of surgery aortic repair was then attempted by cusp extension. Since 2013 the strips have been tailored from extracellular matrix. Results The mean aortic cross-clamp time was 90 ± 32 min. The mean cardiopulmonary bypass time was 126 ± 38 min. There was no in-hospital death. Re-exploration for bleeding was required in 1 patient. During follow-up, 1 patient needed reoperation at 1 year due to endocarditis. All patients remained alive in New York Heart Association (NYHA) functional class I. The echocardiographic findings remained unchanged in all cases during follow-up. Conclusions Our modification of aortic valve repair results in a good outcome.

Late complete atrioventricular block after hybrid perimembranous ventricular septal defect closure in a neonate

Kardiologia Polska Copyright

First Polish analysis of the treatment of advanced heart failure in children with the use of BerlinHeart EXCOR mechanical circulatory support

BACKGROUND The treatment of advanced heart failure (HF) in children and infants poses a serious management problem. Heart failure in that patient group is usually of congenital aetiology. The treatment schedules for paediatric patients are in most cases adapted from the guidelines for treatment of adults. Up to 2009, the treatment of that extremely difficult group of patients was limited to pharmacological therapy and occasional heart transplantations. Constantly increasing problems with recruiting donors, especially for the paediatric group, contribute to the fact that mechanical support with the use of ventricular assist devices is for many children the only chance of surviving the period of waiting for a heart donor. AIM The aim of the study was to analyse the outcomes of circulatory support in Poland and to assess the advisability of this method for treatment of children with severe HF. METHODS This treatment of paediatric patients is currently used in three Polish centres. From December 28, 2009 to August 1, 2015, 27 implantations of BerlinHeart EXCOR® mechanical circulatory support system were performed in children aged from one month to 16 years (10 patients below one year of age; 37%). Left ventricular assist devices were implanted to 21 patients, whereas the remaining children received biventricular support. The most common reason for using this method was HF developed in the course of cardiomyopathy. In one case, HF after Fontan operation was the indication. RESULTS The duration of the circulatory support period ranged from six to 1215 days. It was followed by successful heart transplantations in 10 (37%) patients, in five (18.1%) it resulted in regeneration of the heart, enabling explantation of the device, whereas three children are still waiting for transplantations. Nine (33%) children died during the therapy because of thromboembolic complications. CONCLUSIONS As follows from our data, circulatory support utilising the BerlinHeart EXCOR® system is an effective and promising method used as a bridge to cardiac transplantation, or for regeneration of the myocardium in paediatric patients. In the group of the youngest and the most difficult patients, the method requires close cooperation of the medical and nursing personnel.

Hybrid muscular ventricular septal defect closure in a 4.5 kg infant followed by sildenafil treatment and transcatheter atrial septal defect occlusion

Corresponding author: Michal Galeczka MD, Department of Congenital Heart Defects and Pediatric Cardiology, SMDZ in Zabrze, Medical University of Silesia, Silesian Center for Heart Diseases, 9 Marii Curie-Sklodowskiej St, 41-800 Zabrze, Poland, phone: +48 609 345 681, e-mail: michalgaleczka@gmail.com Received: 12.11.2017, accepted: 10.12.2017. Hybrid muscular ventricular septal defect closure in a 4.5 kg infant followed by sildenafil treatment and transcatheter atrial septal defect occlusion

Implantation of stents for postsurgical recoarctation of the aorta in adolescents and adults

BACKGROUND Results of stent implantation (SI) of postsurgical recoarctation of the aorta (ReCoA) are not frequently published. AIM This study sought to retrospectively evaluate results of SI in ReCoA in older children and adults. METHODS Twenty-eight SIs were performed on 26 ReCoA patients with a median age of 23 (10-65) years. Dependent upon availability, the following stents were applied: Palmaz, Cheatham Platinum (CP), Andrastents XL/XXL (AS), Covered CP (CVCP) stents, and self-expanding stents (Smart). Generally, high-pressure balloons were applied to dilate stents. RESULTS The procedure was effective in 20/26 patients (77.7%). The mean peak systolic gradient reduced from 40.5 ± 18.7 mm Hg to 13.1 ± 12.1 mm Hg (p < 0.05), and the diameter of the stenosed segment increased from 7.5 ± 3.02 mm to 13.1 ± 3.32 mm (p < 0.05). In six cases (including a patient treated with a Smart stent) transaortic pressure gradient after SI remained > 20 mm Hg (stiff postsurgical lesion). For one patient (40-year-old male), an acute dissection of the aorta occurred during balloon predilatation. Immediate CVCP implantation resolved this problem. Two more CVCPs were used - one to close a small aortic aneurysm that appeared five years after a Palmaz SI and another to stabilise a broken CP bare metal stent. There were no deaths or aortic dissections during follow-up, and most patients were able to reduce or suspend their medication for systemic hypertension. CONCLUSIONS Endovascular stenting of ReCoA in adults and adolescents appears to be an acceptable method of treatment in experienced hands. However, for some patients the presence of a stiff lesion can provoke suboptimal results. Considering the serious complications that can occur after SI, all patients should have regular follow-up (including an imaging study). Covered stents should always be available in the cathlab as a rescue device when implanting stents in coarctation of the aorta patients.

Difficult-to-treat asthma and dysphagia in an adult patient with aberrant right subclavian artery.

288 of uncontrolled asthma and to establish a relationship between vascular anomaly and asthma. The patient had a history of mild atopic asthma since childhood. The course of the disease aggravated at the age of 32 years, when she began to suffer from multiple, sudden episodes of shortness of breath, coughing, and wheezing. Within the last 7 years, she required 35 hospitalizations on that account, including 4 hospitalizations in intensive care units. Symptoms persisted despite treatment with high doses of systemic and inhaled corticosteroids. Chest computed tomography (CT) performed 5 years ago owing to suspicion of pulmonary embolism after a lower limb fracture revealed a vascular anomaly in the form of an ARSA. At first, it was not associated with troublesome respiratory symptoms; however, with time, the patient reported increasing difficulty swallowing solid Aberrant right subclavian artery (ARSA), also called arteria lusoria, is the most common congenital abnormality of large vessels branching from the aortic arch, which occurs with a prevalence of 0.5% to 2% of the general population.1 In infants, the disorder can manifest as stridor, wheezing, and recurrent respiratory infections due to the lack of sufficient rigidity of the trachea.1 In children, dyspnea or dysphagia can occur. In adults, this anomaly is usually clinically silent. When symptomatic, it produces dysphagia (dysphagia lusoria).2 The first case of an elderly woman with dysphagia caused by an ARSA was described in 1787 by Bayford.3 The predominance of dysphagia in adulthood may result from increased rigidity of the trachea, progressive atherosclerosis and stiffness of the ARSA, its tortuosity, or aneurysmal dilation.4 We report here a case of a symptomatic ARSA in an adult woman with asthma. A 39-year-old woman was admitted to the Department of Pulmonology to extend the diagnosis

Temporary left ventricular assistance for extreme postoperative heart failure in two infants with Bland-White-Garland syndrome

Anomalous origin of the left coronary artery from the pulmonary artery (Bland-White-Garland syndrome – BWG) is a serious congenital cardiac anomaly leading to myocardial ischemia with severe heart failure. Immediate surgical correction is the treatment of choice, and the risk of postoperative complications depends on the degree of myocardial injury. The authors present two cases of infants with BWG, in whom long-term (175 and 26 days) left ventricular assistance with a Berlin Heart device was used, resulting in successful weaning from the support and subsequent hospital discharge. Because of serious hemorrhagic complications and their neurological consequences observed in the first patient, the anticoagulation protocol was modified in the second patient, providing more stable support and allowing the device to be removed after a shorter period of time. The Berlin Heart left ventricular assist device may be treated not only as a bridge for transplantation but also, considering the shortage of donors in this age group, as a bridge to recovery.