Case report: Cardiac intimal sarcoma in a young child

Undifferentiated mesenchymal tumors from the intimal layer (intimal sarcomas) are rare within the ventricles and exceptional in children. A rare case of an intimal sarcoma located in the right ventricle in a young child is presented with need for urgent surgical resection due to mechanical flow obstruction. Tumor cells showed amplification of MDM2 gene and a homozygous loss of CDKN2A on 9p21. A review of the literature regarding primary cardiac malignancies and intimal sarcoma in children is provided

Cardiac outcome in classic infantile Pompe disease after 13\xe2\x80\xafyears of treatment with recombinant human acid alpha-glucosidase

Background: Cardiac failure is the main cause of death in untreated classic infantile Pompe disease, an inheritable metabolic myopathy characterized by progressive hypertrophic cardiomyopathy. Since the introduction of enzyme replacement therapy (ERT), survival has increased significantly due to reduced cardiac hypertrophy and improved cardiac function. However, little is known about ERT\'s long-term effects on the heart. Methods: Fourteen patients were included in this prospective study. Cardiac dimensions, function, conduction and rhythm disturbances were evaluated at baseline and at regular intervals thereafter. Results: Treatment duration ranged from 1.1 to 13.9 years (median 4.8 years). At baseline, all patients had increased left ventricular mass index (LVMI) (median LVMI 226 g/m2, range 98 to 599 g/m2, Z-score median 7, range 2.4\xe2\x80\x9312.4). During the first four weeks, LVMI continued to increase in six patients. Normalization of LVMI was observed in 13 patients (median 30 weeks; range 3 to 660 weeks). After clinical deterioration, LVMI increased again slightly in one patient. At baseline, PR interval was shortened in all patients; it normalized in only three. A delta-wave pattern on ECG was seen in six patients and resulted in documented periods of supraventricular tachycardias (SVTs) in three patients, two of whom required medication and/or ablation. One patient had severe bradycardia (35 beats/min). Conclusion: This study shows that ERT significantly reduced LVMI, and sustained this effect over a period of 13.9 years. The risk for rhythm disturbances remains. Regular cardiac evaluations should be continued, also after initially good response to ERT

Adverse Outcome of Coarctation Stenting in Patients With Turner Syndrome

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Hybrid stenting of aortic coarctation in very low birth weight premature infant

A very low birth weight infant with severe aortic coarctation developed progressive left ventricular dysfunction and pulmonary overflow with hemorrhage, while receiving prostaglandins. To avoid morbidity from conventional surgery or percutaneous intervention, a two-step strategy was performed at a weight of 970 g. First vascular access was obtained through sternotomy: a 3/8 mm coronary stent was deployed through a 4 French sheath in the ascending aorta; the arterial duct was clipped. At the age of 5 months, the stent was removed and the aortic arch reconstructed with an end-to-end anastomosis through lateral thoracotomy. This strategy was not associated with morbidity typical for premature infants with congenital heart disease.status: publishe

Flow reduction of a neonatal stented arterial duct by covered coronary stent

The final flow rate through a stented duct is variable and depends on stent diameter, ductal length, and ductal tissue protrusion after ductal constriction. Typically after duct stenting, there is initially mild overperfusion which may require some antifailure treatment. Severe heart failure is uncommon, but in some cases flow reduction is required. We present a case of overperfusion after arterial duct stent implantation which was successfully managed with implantation of covered stents. © 2015 Wiley Periodicals, Inc.status: publishe

Transhepatic implant of a trimmed Melody™ valved stent in tricuspid position in a 1-year-old infant

Percutaneous valved stent implantation is precluded in small infants because large delivery sheaths and large devices. We describe a procedure in a 1-year-old boy in whom a 19 mm Epic™ valve in tricuspid position had become dysfunctional. As the internal diameter of the prosthetic valve was about 16 mm, the only available valve was the Melody™ valved stent. Technical modifications were required to address issues like venous access, the bulky delivery system, and the length of the valved stent. The Melody™ valved stent was surgically trimmed and mounted on a 16 mm Tyshak balloon, access was provided transhepatically through a short 18 Fr sheath. After deployment, the intrahepatic route was sealed with two vascular plugs (8 and 10 mm) in tandem. The procedure was uncomplicated with perfect valve function 18 months after implant. © 2016 Wiley Periodicals, Inc.status: publishe

Cracking a tricuspid perimount bioprosthesis to optimize a second transcatheter sapien valve-in-valve placement

Bioprosthetic valves degenerate over time. Transcatheter valve-in-valve procedures have become an attractive alternative to surgery. However, every valve increasingly diminishes the diameter of the valvar orifice. We report a 12-year-old female who had a previous transcatheter tricuspid valve-in-valve procedure; cracking the ring of a Carpentier Edwards Perimount valve by means of an ultrahigh pressure balloon allowed implantation of a further larger percutaneous valve. The advantage of this novel approach permits enlarging the inner valve diameter and may facilitate future interventions and prolong time to surgery. © 2016 Wiley Periodicals, Inc.status: publishe

A custom-made percutaneous flow-restrictor to manage a symptomatic congenital porto-systemic shunt in an infant

Portosystemic shunts allow splanchnic blood to bypass the liver unfiltered, and may cause serious pulmonary and cerebral dysfunction; closure is therefore recommended. In patients where the portal system is hypoplastic, closure by a staged approach with a flow reducer may be necessary. We report a new, reliable, short, and adjustable device that can be delivered through a small 8-Fr sheath.status: publishe

Unsuccessful resuscitation of a preterm infant due to a pneumothorax and a masked tension pneumopericardium

Pneumopericardium is the least common form of air leak in infants. A tension pneumopericardium is even more infrequent but associated with a very high mortality rate. We describe the case of an unsuccessful resuscitation in a preterm infant due to a pneumothorax and tension pneumopericardium. Despite relatively mild pressure ventilation the patient developed massive pulmonary interstitial emphysema. The extra-alveolar air spread from the interstitium towards the mediastinal space (Macklin effect) and caused a pneumothorax and pneumopericardium, which evolved towards a tension pneumopericardium after a traumatic mechanical procedure. The infant deteriorated acutely. Despite prompt pleural drainage there were no signs of recovery at any time. Postmortal examination revealed a tension pneumopericardium and massive interstitial pulmonary emphysema, which was not obvious on radiographical investigation. In cases of acute deterioration of a ventilated neonate, one should always rule out pneumothorax. If the patient does not recover after pleural drainage and cardiac resuscitation a (tension) pneumopericardium should be considered.status: publishe