Combined aortic root and right ventricular outflow tract replacement with mechanical conduits in adult patients after repeated surgery for congenital heart disease.

OBJECTIVE: To manage aortic root aneurysms and right ventricular outflow tract (RVOT) graft dysfunction in adult patients after repeated surgery for congenital heart disease, we performed combined prosthetic aortic root and RVOT replacement. METHODS: The procedure was performed in 5 patients (2 truncus arteriosus, 2 variants of tetralogy of Fallot, and 1 congenital aortic stenosis), aged 23, 24, 27, 29, and 34 years, who presented with progressive dilation of the aortic root and aortic regurgitation as well as RVOT graft dysfunction. All patients had undergone a median of 3 previous operations and this procedure was their third (in 1), fourth (in 3), or fifth (in 1) operation. The mean interval since the previous operation was 8.2 (3-16) years. RESULTS: Mean cardiopulmonary bypass (CPB) and aortic cross-clamping (AXC) times were 354 (248-422) and 113 (69-142) minutes, respectively. One patient died on the 16th postoperative day from respiratory failure caused by pulmonary bleeding. The other four patients survived the operation and are in New York Heart Association functional class II or less at a maximum of 41 months follow-up. Mechanical valve function in the aortic and pulmonary position is good without any thromboembolic or bleeding complications in all surviving patients. CONCLUSIONS: Combined aortic root and RVOT replacement with mechanical conduits in adult patients after repeated surgery for congenital heart disease is a complex operation requiring long CPB time. However, this procedure has the potential to avoid a predictable reoperation associated with conventional biological graft replacement.

Neonatal Kawashima intraventricular repair for Taussig-Bing anomaly with oblique relationship of the great arteries.

An arterial switch operation is considered the procedure of choice for the repair of Taussig-Bing anomaly, because it can be performed in most patients. However, after taking problems such as neoaortic insufficiency or myocardial perfusion disturbances after the arterial switch operation into account, intraventricular repair might be reconsidered as an option in selected patients. We present a case of Taussig-Bing anomaly in a patient with an oblique relationship of the great arteries, which was successfully managed by Kawashima intraventricular repair in the neonatal period, and discuss the feasibility of this option with a review of the literature.

Coexistent cardiac rhabdomyoma with mitral valve anomaly in patients with tuberous sclerosis: a case report.

Cardiac rhabdomyoma is frequently associated with tuberous sclerosis. However, very few cardiac malformations have been described with tuberous sclerosis. We report a rare case of coexistent cardiac tumor with mitral valve anomaly in a patient with tuberous sclerosis, who developed mitral regurgitation and required a surgical procedure with histological confirmation of cardiac rhabdomyoma.

[Usefulness of a VDD defibrillation electrode in recording atrial electrograms during atrial flutter and atrial fibrillation]. / Nutzen einer VDD-Defibrillationselektrode zur Aufzeichnung von atrialen Elektrogrammen bei Vorhofflimmern/-flattern.

BACKGROUND: Monitoring of atrial signals improves the accuracy in identifying supraventricular tachyarrhythmias to prevent inappropriate therapies in patients with implantable cardioverter-defibrillators (ICD). Since complications due to the additional atrial lead were found in dual chamber ICD systems with 2 leads, we designed a single-pass VDD-lead for use with dual chamber ICDs. PATIENTS AND METHODS: After promising animal experiments in a German multicenter study a prototype VDD lead (single-coil defibrillation electrode with 2 additional fractally coated rings for bipolar sensing in the atrium) was temporarily used in 20 patients. Atrial and ventricular signals were recorded during sinus rhythm, atrial flutter, atrial fibrillation and ventricular tachycardia or ventricular fibrillation. Terminations of ventricular arrhythmias were performed by internal DC shock. RESULTS: The implantation of the electrode was successful in 18 of 20 patients. Mean atrial pacing threshold was 2.45 +/- 0.9 V/0.5 ms, mean atrial impedance was 215 +/- 31 Ohm. Atrial amplitudes were greater during sinus rhythm (2.7 +/- 1.6 mV) than during atrial flutter (1.36 +/- 0.28 mV, p < 0.05) or atrial fibrillation (0.92 +/- 0.29 mV, p < 0.01). During ventricular fibrillation atrial "sinus"-signals had significantly (p < 0.01) lower amplitudes than during sinus rhythm. Mean ventricular sensing was 13.3 +/- 7.9 mV, mean ventricular impedance was 577 +/- 64 Ohm. Defibrillation was successful with 20 J shock. 99.6% of P waves could be detected in sinus rhythm and 85 +/- 9.9% of flutter waves during atrial flutter. During atrial fibrillation 55% of atrial signals could be detected without modification of the signal amplifier. CONCLUSIONS: A new designed VDD dual chamber electrode provides stable detection of atrial and ventricular signals during sinus rhythm and atrial flutter. For reliable detection of atrial fibrillation modifications of the signal amplifier are necessary.