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Pentalogy of Cantrell (PC) is an extremely rare multiple congenital anomaly syndrome, characterized by the presence of five major malformations: midline supraumbilical abdominal wall defect (which results in omphalocele), lower sternal defect, diaphragmatic pericardial defect, anterior diaphragmatic defect and various intracardiac malformations (mostly ventricular septal defect) [1]. Additionally, although not part of the classic PC, some severe cases have been associated with herniation of the heart through the diaphragmatic defect, resulting in ectopia cordis (EC) [2]. Such a diagnosis carries a disastrous prognosis without surgical correction or palliation [3]. Furthermore, miscellaneous intracardiac abnormalities even worsen the survival. Multiple chest interventions and complex anatomy impede their surgical treatment; therefore, the transcatheter approach may be the only possibility. According to our best knowledge, no percutaneous atrial septal defect (ASD) closure has been reported in a patient with PC.

Ventricular septal defects closure (VSD) depending on the anatomy and clinical setting can be performed surgically or by a hybrid and transcatheter approach. Two cases of children with VSD will be presented. Patients' defects were closed with various types of occluders made of nitinol wire mesh occluder, patent ductus arteriosus (PDA) type. The first case was a 2.5-year-old boy after cardiosurgical correction of tetralogy of Fallot (TOF). After the procedure, a significant haemodynamic residual VSD was observed, which was not successfully closed during the subsequent reoperation. Despite pharmacological treatment, symptoms of heart failure were observed in this patient. In echocardiographic images the residual VSD was presented as a tunnel-like dissection of the ventricular septum (length 6 mm and diameter 3.4 mm). The defect was closed via arterial access with an Amplatzer Duct Occluder II (ADO II). The procedure was successfully performed without any medical complications. In this child, a significant shunt reduction and a noticeable improvement in the patient's clinical status and diminished symptoms of heart failure were noticed. The second patient was a 4-year-old girl suffering from a multi-perforated perimembranous VSD accompanied by a ventricular septal defect with aneurysm. The defect was closed by a venous approach with a PDA Cardio-O-Fix occluder (very similar to ADO I). No short-term or long-term complications were visible during or after the procedure. Only a mild residual shunt through the VSD was observed 6 months afterwards. Transcatheter VSD closure with a proper morphology, with occluders of type Amplatzer Duct Occluder ADO I or ADO II, constitutes a safe and effective therapeutic alternative.

Patent ductus arteriosus (PDA) is one of the most prevalent congenital heart diseases. Transcatheter closure of PDA is the treatment method of choice. In spite of recent advances in transcatheter management, the occlusion of certain anatomical types of PDA remains a challenge. The aim of the study was to report novel use of the Amplatzer Vascular Plug type II (AVPII) for closure of large elongated type of PDA (type E according to Krichenko classification) in a 19-year-old man. In clinical examination a soft continuous murmur was heard in the 2-3 left intercostal space. Echocardiography confirmed left to right shunt through the PDA. Left ventricle and left atrium were at the upper limit for body weight. In angiography the duct was 20 mm long, 7 mm wide with 3 mm narrowing at the pulmonary end. For the procedure we applied a 12 mm AVPII. The AVPII is a self-expanding, nitinol wire mesh occluder dedicated for arterial and venous occlusion in the peripheral circulation. It was delivered through a 6 F catheter retrogradely. The distal disc and medial part of the AVPII were opened inside the duct, and the proximal disc in the pulmonary artery. Complete closure of the PDA was confirmed in angiography. No complications during the procedure or 3-month follow-up were observed. Application of the AVPII is a safe and effective method of treatment for adult patients with elongated type of PDA.

We describe a patient who, at the age of 6 months (body weight 6.1 kg), was diagnosed with a large atrial septal defect (with diameter 10–11 mm) that had spontaneously closed in a 1-year period. The timing of transcatheter intervention in children should be considered individually, paying close attention to subsequent echocardiographic data.

Two cases are presented. In the first patient (8-year-old boy) after Glenn operation without closure of pulmonary outflow from the common ventricle (SV-PA) despite transcatheter SV-PA closure increased mean pulmonary artery pressure (mPAP) (22 mm Hg) remained. After 6 months of sildenafil therapy he was catheterized again. His mPAP dropped to 10 mm Hg and a Fontan operation was subsequently performed. The second patient was a 25-year-old man, 20 years after a Fontan operation, presenting protein los ing enteropathy and severe heart failure. All syndromes diminished significantly after medical therapy with sildenafil. Good clinical results of sildenafil therapy were maintained after 1 and 4 years of treatment. Chronic therapy with sildenafil can be beneficial in patients before and after the Fontan operation with elevated pulmonary artery pressure.

Paravalvular leak (PVL) is usually related to disruption of prosthetic valve sewing ring sutures. Many previous reports have described transcatheter paravalvular leak closure in adult patients [1–4], but those describing such a procedure in children are scarce. A 16-year-old boy underwent an aortic valve replacement (AVR) with a stented bioprosthesis Hancock 23 mm due to combined aortic stenosis and insufficiency. Soon after, a significant paravalvular leak was detected and initially managed medically. However, within the next 5 months the patient became breathless on exertion and developed significant left ventricular dilatation. Paravalvular leak closure was performed under general anesthesia, with transesophageal echocardiographic (TEE) and angiographic guidance. Aortography confirmed grade 3 aortic regurgitation (Figure 1A). The paravalvular leak size was evaluated in TEE at 4 × 3 mm. It was identified as posterior, from the noncoronary Valsalva sinus into the left ventricle (LV). The defect was easily crossed using two JR catheters and hydrophilic wires, then Amplatz extra-stiff guidewires were introduced from both femoral arteries into the LV. Over these, 120 cm long sheaths (6 Fr and 7 Fr) were advanced through the paravalvular leak. No obstruction during the crossing was met, which was considered confirmatory of the defect size exceeding what was expected from TEE. Accordingly, the decision was taken to implant 3 devices in order to avoid a residual shunt. Two 8/4 Vascular Plugs type III were implanted and one 6/3 mm Vascular Plug type III (St Jude Medical Inc.). After sequential opening of the distal discs in the LV all plugs were withdrawn simultaneously to the level of the paravalvular leak orifice. The proximal discs were then opened (Figure 1B) with immediate complete closure of the shunt on TEE (Figure 1C). Prior to devices release, proper function of the prosthetic valve was confirmed by TEE. Control aortography showed proper position of devices without Ao–LV shunt (Figure 1D). Fluoroscopy time was 18.5 min. The next morning the patient underwent transthoracic echocardiography which confirmed a good procedure result with normal function of the prosthetic aortic valve, without any regurgitant jet. The patient was discharged 3 days after the procedure on 150 mg Aspirin q.d. Figure 1 A – Aortography RAO 30 Caud 30. Paravalvular leak from posterior Valsalva sinus to LV. B – Three Amplatzer Vascular Plug 3 devices closing paravalvular leak still connected with delivery system. C – Transesophageal echocardiographic ... Early mortality in redo AVR reaches 3.5–6% [1]. Transcatheter treatment has been constantly developing during the last years. Transcatheter PVL closure was initially performed with devices dedicated to patent ductus arteriosus, ventricular septal defect and atrial septal defect [2]. In our and others’ opinion, especially useful for such purposes is Amplatzer Vascular Plug type III [3]. Lack of internal fabric, which could potentially promote clot formation, may be considered a major disadvantage of the device. We suspect that use of one or two bigger plugs in the hereby-presented case might result in residual leaking and possibly hemolysis [4]. To avoid such complications we chose 3 plugs to ensure tight as possible filling of the defect. Simultaneous implantation of multiple devices increases the chance of final success, which was described previously [3].

The 12th annual Conference of Pediatric Cardiology Section organized in Zabrze in 7–8.06.2013 was a very important medical event for cardiologists and doctors looking after patients with congenital heart diseases (CHD). The symposium was part of the 20th Jubilee of the International Cardiology Conference traditionally taking place in Zabrze in spring. Almost 2000 doctors including 300 pediatricians from all parts of Poland participated in this event. The main topic of our conference was advances in interventional cardiology of CHD and was presented in the 5 following sessions. In the opening session interesting introductory lectures were presented by professors W. Kawalec, B. Brzezinska-Rajszys and B. Maruszewski from IPCZD in Warsaw. The second session concerned standard percutaneous interventions in treatment of selected CHD. Angioplasty and stent implantations in coarctation of aorta (CoA) were excellently described by Dr. M. Zubrzycka, closure of atrial septal defects by Prof. M. Szkutnik, closure of patent arterial ducts (PDA) by Dr. K. Godlewski, pulmonary balloon valvuloplasty by Dr. P. Werynski and balloon aortic valvuloplasty (BAV) by Prof. J. Bialkowski. In the third session titled “New challenge for interventional cardiology” lectures were presented by Prof. J. Szymkiewicz-Dangel (fetal interventions), Dr. P. Dryzek (neonatal interventions), Dr. I. Haponiuk (hybrid procedures), Dr. R. Fiszer (percutaneous pulmonary valve implantation), Dr. T. Moszura (interventions in univentricular heart) and Dr. R. Sabiniewicz (closure of vascular malformations). In the next session Prof. K. Bieganowska, Prof. W. Bobkowski and Dr. R. Lenarczyk presented in their lectures summaries concerning advances in electrotherapy of rhythm and conduction disturbances in children (pacemaker and cardioverter implantations as well as radiofrequency ablation and electroanatomical 3D mapping). The last, probably the most interesting session was entitled “What is new in the world? Invited experts lectures”. The first speaker was Prof. Shiliang Jiang, leader of the biggest cardiological center in China (Fuwai Hospital, Beijing). This hospital was visited recently by a Polish government delegation with Marshall Ewa Kopacz. Prof Jiang presented Chinese experience in percutaneous closure of ventricular septal defects (VSD). There were more than 20 000 patients with VSD treated percutaneously in all over China (for comparison the biggest multicenter studies in Europe published by M. Carminati [1] enrolled 450 cases). In the Chinese study perimembranous VSDs were closed initially by US implants (asymmetric Amplatzer devices), and later by Chinese, domestic occluders modified by Prof. Qin from Shanghai [2]. The results presented by Prof. Jiang indicated that the procedures were highly successful with no major complications. Complete AV block in follow-up was noted in less than 1% of cases – in comparison to 5% of cases in M. Carrminati's study [1]. Some doubts were expressed by the chairmen (Prof. W. Ruzyllo and Prof. G. Brzezinska) and concerned age of the percutaneously treated patients (median – 8 years). It was surprising as indications for VSD closure are usually much earlier. The second speaker was Prof. Felix Berger – head of the Pediatric Cardiology Ward in Charite Hospital in Berlin. He gave an outstanding lecture concerning indications, effectiveness and long-term follow-up of BAV in children and teenagers. His conclusions were similar to my earlier presentation in the previous session. The main conclusion was the statement that BAV in children although palliative in nature was the treatment of choice in selected patients, helping to delay aortic valve replacement. Prof. Berger mainly referred to the recently published BAV results of 20 pediatric cardiology centers from Germany, Austria, and Switzerland [3]. According to that study performed in a group of 1004 patients treated with BAV, 70% did not require cardiac surgery up to 10 years after the initial procedure. The next lecturer, Dr. Gejun Zhang – head of the Hemodynamic Laboratory in Fuwai Hospital in Beijing – showed us his experience of 23 procedures of percutaneous closure of ruptured Valsalva aneurysm (our own material unique in Europe enrolled 13 patients treated by that method). The next speaker was Dr. Christian Juxt (head of the cath lab in Munster – Germany). He presented new stents for dilation of great vessels (mostly CoA), mentioned the encouraging results of applications of cobalt–chromium stents (namely Andrastents) and expressed his negative opinion about the Advanta V12 stent (similar to the impressions of Dr. L. Benson and Dr. J. Di Giovanni). According to their opinion, the Advanta V12 stent although doubly covered has minor radial strength which can be responsible for stent deformation and dislocation. There were also two very interesting lectures of guests from Spain. Dr Fernando Ballesteros (from Gregorio Maranon Hospital in Madrid) talked about his center experience in closing PDA in preterm neonates (biggest material in the world) and Dr Fernando Rueda (from La Coruna Hospital) presented new opportunities in application of rotational angiography in diagnostics and treatment of CHD. On the next day in two stimulating sessions there were presented and discussed 22 casuistic cases almost from all pediatric cardiology centers from Poland. Two of them traditionally were chosen as the most interesting: the first case from Poznan (presenter – R. Surmacz): “Percutaneous closure of vascular fistula between right ventricle and right atrium” and the second from Zabrze (presenter – J. Rycaj): “Effectiveness of corticoids in fulminant myocarditis – case report”. In summary, in my opinion the conference was very successful. The vivid discussions were particularly important but they were limited because of the shortage of time caused by the rich program.