1-year left ventricular assist device (LVAD) experience as bridge to heart transplantation in an infant with Bland-White-Garland syndrome.

Left ventricular assist device (LVAD) implantation has become an established therapy in adults as well as in children as a bridge to heart transplantation or to aid myocardial recovery. We describe the first case worldwide of an infant suffering from Bland-White-Garland syndrome successfully treated with a left ventricular assist device (Berlin Heart(R); Excor(R) Pediatric) as a bridge to heart transplantation for a period of more than one year.

[Atrial flutter after orthotopic heart transplantation due to recipient-to-donor transatrial conduction]. / Vorhofflattern in der Frühphase nach orthotoper Herztransplantation.

A few weeks after orthotopic heart transplantation, a male adolescent developed atrial arrhythmias of the donor heart due to an atypical recipient atrial flutter with a recipient-to-donor transatrial conduction resulting in an absolute arrhythmia. Under medication with propafenone, the atrial flutter of the donor heart could be terminated with cardioversion.

A decade of staged Norwood palliation in hypoplastic left heart syndrome in a midsized cardiosurgical center.

Hypoplastic left heart syndrome (HLHS) is a challenge for the pediatric cardiologist and the surgeon. It is generally assumed that the postoperative outcome after surgery for congenital heart disease is influenced by the institutional size. We present the results of 43 patients with true HLHS (situs solitus and atrioventricular and ventriculoarterial concordance) referred for operation between 1992 and 2002 in our center. Two children had atrioseptostomy: one died soon after the operation, and the other one was transplanted successfully but died at the age of 6 months following acute rejection. The remaining 41 underwent Norwood I palliation, 21 stage II palliation, and 10 stage III palliation. Early mortality was 29% after stage I operation, 4.7% after stage II palliation, and 0% after stage III operation. Overall mortality was 39% after stage I, 9.5% after stage II, and 10% after stage III operation. Low birth weight was associated with a higher mortality (p < 0.05). Mortality declined with increasing experience, comparable to the results of very large cardiosurgical centers with many more patients. The quality of surgery and perioperative management in smaller pediatric cardiosurgical centers can reach the level of very large centers.

Effective systolic orifice area of the aortic valve: implications for Doppler echocardiographic cardiac output determinations.

BACKGROUND: Substantial research using echocardiography has established that stroke volume (SV) or cardiac output (CO) can be measured non-invasively at the level of the aortic valve (AV) with high accuracy. Stroke volume is the product of the velocity time integral occurring at the sampling site and the effective systolic AV orifice area (AVOAeff). Nevertheless, a generally accepted method for the determination of AVOAeff is still lacking. METHODS: Aortic valve OAeff was measured in 228 consecutive patients scheduled for coronary artery surgery. Two widely adopted methods were applied to approximate the constantly changing orifice area of the AV: (1) the circular orifice model (AVOA-CM), and (2) the triangular orifice model (AVOA-TM). Aortic valve OA-CM assumes the shape of a circle as an appropriately time averaged geometrical model, and AVOA-TM takes the shape of an equilateral triangle for granted. RESULTS: The AV was easily imaged by echocardiography in both short- and long-axis views in all patients. Relying on AVOA-CM, AVOAeff was 3.49+/-0.77 cm2. AVOA-TM estimates were 2.80+/-0.55 cm2 (mean+/-SD). The results did not agree (bias analysis). CONCLUSIONS: The echocardiographic measurement of SV or CO at the level of the AV has to be reconsidered.

Cardiac transplantation in neonatal Marfan syndrome -- a life-saving approach.

Marfan syndrome is a connective tissue disease with typical clinical signs and cardiac involvement. Its appearance in the neonatal period has a bad prognosis due to incompetence of all cardiac valves with subsequent congestive heart failure. Conservative management usually fails, the children die during their first year of life. We report on a girl with neonatal Marfan syndrome who suffered from regurgitance of all cardiac valves, enlarged ventricles, and dilated great arteries. She was NYHA class IV. At the age of six months she underwent heart transplantation. To prevent aneurysm formation and dissection of the great vessels, the whole aortic arch and pulmonary trunk were replaced as well.

Long QT syndrome and life threatening arrhythmia in a newborn: molecular diagnosis and treatment response.

Intrauterine and neonatal manifestations of congenital long QT syndrome are associated with a high cardiac risk, particularly when atrioventricular block and excessive QT prolongation (> 600 ms(1/2)) are present. In a female newborn with these features, treatment with propranolol and mexiletine led to complete reduction of arrhythmia that was maintained 1.5 years later. High throughput genetic analysis found a sodium channel gene (LQT3) mutation. Disappearance of the 2:1 atrioventricular block and QTc shortening (from 740 ms(1/2) to 480 ms(1/2)), however, was achieved when mexiletine was added to propranolol. This effect was considered to be possibly genotype related. Early onset forms of long QT syndrome may benefit from advanced genotyping.

Congenital aortic regurgitation due to absent aortic cusps and high-degree mitral stenosis.

Congenital absence of aortic cusps leads to severe aortic regurgitation. We present a newborn with this rare entity with extreme mitral stenosis. Hemodynamic features were those of hypoplastic left heart syndrome. Surgical management consisted of initial modified Norwood procedure followed by orthotopic heart transplantation.

Mycotic cerebral vasculitis in a paediatric cardiac transplant patient excludes misadventure.

We present the case of a 10-year-old girl with cardiomyopathy who received a heart transplant. Due to organ rejection, the dosage of immunosuppressive agents was increased postoperatively. The patient complained of intermittent headaches in the following days and developed a haemorrhagic necrosis of the left thalamus. A week later, an oral dose of cyclosporin A was accidentally given intravenously, and 2 weeks later a recurrent subarachnoid haemorrhage of unknown origin was diagnosed. The clinical course was then characterised by progressive deterioration resulting in coma, fluctuating brain stem symptoms and the development of a massive cerebral oedema with subsequent brain death. A coroner's autopsy was instigated to investigate a claim of medical misadventure. Neuropathological investigations found a focal infiltration of fungal hyphae in the left posterior cerebral artery resulting in necrosis of the vascular wall and thus explaining the source of the recurrent subarachnoid haemorrhage which eventually resulted in the girl's death. Medical misadventure due to the administration of cyclosporin was not directly responsible for the death of this patient. This case illustrates that it is of paramount importance to copiously sample and investigate the basal cerebral arteries in cases of subarachnoid haemorrhage of unknown origin, in particular in a medico-legal context.

Cardiac leiomyosarcoma of the right atrium in a teenager: unusual manifestation with a lifetime history of atrial ectopic tachycardia.

A 16-year-old girl presented with atrial fibrillation. Transesophageal echocardiography revealed a right atrial leiomyosarcoma. Her past medical history was remarkable for incessant atrial ectopic tachycardia (AET) beginning in early infancy and continuing throughout childhood and adolescence that was refractive to medical and nonpharmacological treatment. After combined surgical and medical therapy, normal sinus rhythm was restored and the patient is currently in complete remission with no recurrent symptoms or atrial arrhythmias at 31 months after surgery and 23 months after the discontinuation of chemotherapy. Atrial tachycardia may be the first, and for prolonged periods, the only manifestation of a cardiac tumor and should prompt thorough investigation of its underlying morphological substrate.

Interaction of fibrinolysis and prothrombotic risk factors in neonates, infants and children with and without thromboembolism and underlying cardiac disease. a prospective study.

UNLABELLED: To evaluate the role of plasminogen activator inhibitor-1 (PAI-1) and tissue-type plasminogen activator (t-PA) in children with an estimated risk of vascular occlusion reported to range from 7% to 16%, we conducted a prospective study in infants and children with underlying cardiac disease. One hundred and twenty-five children (neonate - 16 years) were investigated. In 9 infants out of the 125 children vascular occlusion occurred, closely related to cardiac catheterisation and arterial or venous lines during major cardiac surgery. Six of the nine neonates and infants with (n=6) and without (n=3) prothrombotic risk factors showed evidence of a basically impaired fibrinolytic system. Five of the nine infants showed increased PAI-1 clearly correlated to the 4G/4G genotype of the plasminogen activator-1 promoter polymorphism along with elevated t-PA concentration before the first diagnostic cardiac catheterisation was performed. One infant presented with increased t-PA concentration only. Five of the six children with reduced fibrinolytic capacity had further prothrombotic risk factors. CONCLUSION: Data of this study indicate that neonates and infants with underlying cardiac disease and basically increased PAI-1 due to the 4G/4G variant of the PAI-1 promoter polymorphism along with elevated t-PA levels in combination with further prothrombotic risk factors are at high risk of developing early thromboembolism during cardiac catheterisation.

Neonatal mechanical bridging to total orthotopic heart transplantation.

BACKGROUND: Until recently, newborns with medically intractable cardiac failure caused by congenital malformations were mostly doomed to death because of the severity of the disease, which precludes a palliative operation, or because of fatal deterioration before availability of a suitable donor heart. METHODS: The recently developed paracorporeal pneumatically driven Medos HIA ventricular assist device offers a therapeutic option for these small infants because it is manufactured in various sizes and is even suitable for cardiac assistance in neonates with a body surface area less than 0.3 m2. RESULTS: We report our initial experience with this device, which we used for univentricular bridging to total orthotopic cardiac transplantation in 3 infants. The device was inserted to support the left ventricle in two instances and to support the right heart in one. Successful bridging to transplantation was achieved in 2 infants for periods of 2 and 7 weeks. CONCLUSIONS: Our experience demonstrates the feasibility of univentricular mechanical support followed by successful cardiac transplantation in infants and newborns.

[A rare combination of partial trisomy 9 with pulmonary atresia]. / Eine seltene Kombination einer partiellen Trisomie 9 mit einer Pulmonalatresie.

We report of an previously unpublished combination of partial trisomy 9 and a membranous pulmonary atresia with a large conotruncal ventricular septal defect. The dystrophic female, term newborn presented after delivery with microcephaly, prominent nose and several other facial and skeletal deformities. The echocardiography and angiography showed a membranous pulmonary atresia with ventricular septal defect. Chromosomal analysis revealed a partial trisomy of the short arm with parts of the long arm of chromosome 9 and a small part of the long arm of chromosome 4. A surgical repair of the heart defect was not performed by the known high risk of severe mental retardation of partial trisomy 9. The child died at the age of six months.

Morphologic characterization and assessment of mitral regurgitation after repair of atrioventricular defects in children.

Severe postoperative mitral regurgitation renders information on the underlying mechanism before reoperation very important, as a potential for mitral valve reconstruction may facilitate the decision whether to reoperate, especially in the very young. This study compares the efficacy of transthoracic echo-cardiography (TTE) and left-ventricular angiography with that of transesophageal echocardiography (TEE) for detection of the mechanism underlying mitral regurgitation and its quantitative assessment in children after repair of common atrioventricular septal defect. Five children aged 1.5 to 16 years were evaluated by TTE, TEE, and angiography for postoperative mitral regurgitation 1 to 21 months after initial repair. TEE showed septal detachment of the mitral leaflet in four patients and reopening of the mitral cleft in one patient as the cause of mitral regurgitation whereas TTE failed in four and angiography in all patients. TEE allows definite identification of morphologic characteristics of mitral regurgitation and reliable assessment of its severity. Thus redo surgery may be safety performed on the bases of TEE findings alone without confirmation by cardiac catheterization.