Aorto-ventricular tunnel

Aorto-ventricular tunnel is a congenital, extracardiac channel which connects the ascending aorta above the sinutubular junction to the cavity of the left, or (less commonly) right ventricle. The exact incidence is unknown, estimates ranging from 0.5% of fetal cardiac malformations to less than 0.1% of congenitally malformed hearts in clinico-pathological series. Approximately 130 cases have been reported in the literature, about twice as many cases in males as in females. Associated defects, usually involving the proximal coronary arteries, or the aortic or pulmonary valves, are present in nearly half the cases. Occasional patients present with an asymptomatic heart murmur and cardiac enlargement, but most suffer heart failure in the first year of life. The etiology of aorto-ventricular tunnel is uncertain. It appears to result from a combination of maldevelopment of the cushions which give rise to the pulmonary and aortic roots, and abnormal separation of these structures. Echocardiography is the diagnostic investigation of choice. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks gestation. Aorto-ventricular tunnel must be distinguished from other lesions which cause rapid run-off of blood from the aorta and produce cardiac failure. Optimal management of symptomatic aorto-ventricular tunnel consists of diagnosis by echocardiography, complimented with cardiac catheterization as needed to elucidate coronary arterial origins or associated defects, and prompt surgical repair. Observation of the exceedingly rare, asymptomatic patient with a small tunnel may be justified by occasional spontaneous closure. All patients require life-long follow-up for recurrence of the tunnel, aortic valve incompetence, left ventricular function, and aneurysmal enlargement of the ascending aorta

Iatrogenic Aortopulmonary Fistula Occurring After Pulmonary Artery Balloon Angioplasty: A Word of Caution.

Abstract We describe the natural history of an adolescent patient who developed an aortopulmonary fistula (APF) after transcatheter stent placement for left pulmonary artery stenosis after neonatal repair of d-transposition of the great arteries. Due to its rarity, the APF was not initially diagnosed and treated until 4 months later. The APF was occluded with a covered stent. Because this is not an isolated report in the literature, we believe it should be considered as a potential complication in all patients after balloon dilation of stenotic pulmonary artery branches after arterial switch surgery

Left ventricular mass after treatment with chemotherapic drugs

A 12-year-old asymptomatic child was urgently admitted to the hospital after a calcified mass in the left lower hemithorax was incidentally detected on a plain chest roentgenogram during routine follow-up (Fig 1A, arrow). He had been previously treated for 24 months with chemotherapic agents injected through a permanent central venous line due to stage III non-Hodgkin\u2019s lymphoma. No atrial mass was visible by twodimensional echocardiography 12 months before, at the time of the central venous catheter removal. A diagnosis of an intracardiac, S-shaped calcified mass, located at the junction between the inferior vena cava and the sinus portion of the right atrium, was achieved by transesophageal two-dimensional echocardiography (Fig 1B, arrow). The calcified mass was successfully removed together with the implantation base with the aid of cardiopulmonary bypass, bicaval cannulation, moderate hypothermia, and cardioplegic arrest (Fig 2A). Postoperative course was uneventful, the child was discharged home, and no atrial mass recurrence was found at a 6-month follow-up. Surgical pathology examination revealed that the rockhard mass consisted of abundant calcific deposits in a hyaline stroma and was attached over an otherwise normal myocardium of the right atrial wall (Fig 2B, RAW right atrial wall; M mass). All these features are in keeping with a calcified mural thrombus, as a possible consequence of previous long-term central intravenous therapy

Heart transplantation in pediatric age

J Cardiovasc Med (Hagerstown). 2007 Jan;8(1):67-71. Heart transplantation in pediatric age. Milanesi O, Cerutti A, Biffanti R, Salvadori S, Gambino A, Stellin G. Source Department of Pediatrics, University of Padova School of Medicine, Padova, Italy. [email protected] Abstract Heart transplantation, formerly the final option for terminally ill children, has now become the treatment of choice for a number of serious acquired or congenital cardiac conditions, which cannot be treated conservatively. Nevertheless, several problems remain unsolved. First of all the shortage of donors, mainly in the first months and years of life, which has become more and more significant with time, regardless of the country, religious belief or culture of the people. Secondly, the long-term impact of immunosuppression in a developing organism, and its possible inter-relation with the primary disorder, which leads to intractable heart failure. Whether a heart transplant is a cure or an ongoing disease for both the child and the family is another matter of concern. These and other topics are covered in this article. PMID: 17255820 [PubMed - indexed for MEDLINE

Systolic and diastolic performance late after the Fontan Procedure for a single ventricle and comparison of those undergoing operation at 12 months of age

To evaluate whether surgical history can influence systolic and diastolic properties of a functional single left ventricle after Fontan operation, we echocardiographically investigated 31 patients (mean age 93.7 months; range 21 to 276); 21 patients were >12 months of age (group A) and 10 were <12 months of age (group B) at the time of the Fontan and/or cavopulmonary procedure. In group A we found persistent abnormalities of left ventricular mass index (95.9 vs 64.1 g/m(2), p <0.05) at long-term follow-up, whereas group B had normal left ventricular mass (61.9 vs 64.1 g/m2, p = NS). In contrast, a diastolic pattern characterized by augmented late diastolic filling was present in both patient groups regardless of age at operation and length of follow-up (E/A in group A 1.3 +/- 0.4, E/A in group B 1.6 +/- 1.5, E/A in controls 1.7 +/- 0.6; A vs B, p = NS; A vs controls, p <0.05). We concluded that patients with a single left ventricle who undergo an unloading procedure performed within the first year of life have complete normalization of left ventricular mass, although a diastolic filling pattern suggestive of augmented compliance persists, regardless of the age at operation