Systemic plasma vascular endothelial growth factor levels as a marker for increased angiogenesis during the single ventricle surgical pathway.

Cyanosis and the cavopulmonary anastomosis (CPA) are associated with pulmonary arterio-venous malformations (PAVMs) in single ventricle physiology. Vascular endothelial growth factor (VEGF) may be a marker of abnormal angiogenesis in this setting. Plasma VEGF levels were measured in 14 patients undergoing the surgical pathway leading to total cavopulmonary connection (TCPC). Venous blood samples were taken before and then months after CPA (n=6), and immediately before TCPC and 1 month thereafter (n=9). Corresponding arterial saturations were correlated with VEGF levels at each time frame. In six patients, pre-CPA plasma VEGF levels rose from a mean of 24.4-112.4 pg/ml (p<0.03) just prior to completion of TCPC. In nine patients, VEGF levels diminished from 115.7 to 48.9 pg/ml (p<0.05) after TCPC. VEGF levels were disproportionately elevated to arterial saturations most notably after CPA (r2=0.002), suggesting an additional angiogenic stimulus besides cyanosis. Plasma VEGF levels fluctuate during the single ventricle surgical pathway, with maximal levels after CPA, and regression after completion of TCPC. High VEGF levels are disproportionate to hypoxia after CPA, potentially incriminating the absence of hepatic flow to the lungs as an abnormal angiogenic stimulus. Measuring VEGF in venous blood may serve as a biochemical marker of angiogenesis after CPA.

Twenty-five years' experience with the arterial switch operation.

OBJECTIVE: The arterial switch operation was introduced in 1976 to correct transposition of the great arteries and has since replaced atrial palliation. This study examines the long-term outcomes of the arterial switch operation. METHODS: Included in the study were 195 patients who underwent the arterial switch operation from 1977 through June 2000. Patients were evaluated for functional class, pulmonary stenosis, left ventricular function, arrhythmias, aortic sequelae, and coronary disease. RESULTS: Overall perioperative mortality was 15%, but rates dropped in the last 5 years to 4% for complex transposition and 0% for simple transposition. Of 151 survivors, 2 died late; 1 died of persisting pulmonary hypertension and 1 died of ventricular fibrillation after coronary pathologic changes. At last follow-up, a total of 145 patients were in New York Heart Association functional class I and 4 patients were in class II. The most frequent complication was pulmonary stenosis, necessitating 45 reinterventions in 26 patients. Left ventricular dysfunction was noted in 5 patients. Arrhythmias were seen in 5 patients: 2 patients had ventricular fibrillation (1 died and 1 required a defibrillator implantation), 1 had sick sinus syndrome, 1 had atrial flutter, and 1 had a single attack of supraventricular tachycardia. Aortic valve incompetence was absent or trivial in 146 patients, 3 had mild incompetence, 1 had moderate incompetence, and 1 had severe incompetence. Coronary sequelae were found in 5 of the 61 patients who underwent angiography. CONCLUSION: Long-term clinical outcome of the arterial switch operation is good, and perioperative mortality is now low. Morbidity is dominated by pulmonary stenosis and coronary artery disease, with the potential for lethal arrhythmias.

Lactacidosis in the neonate is minimized by prenatal detection of congenital heart disease.

OBJECTIVES: To investigate the impact of prenatal detection of congenital heart disease on preventing severe preoperative lactacidosis. DESIGN: Patients operated upon for congenital heart disease during the first 31 days of life (n = 209) were studied retrospectively, 21 were diagnosed prenatally and 188 patients had not been diagnosed prenatally. Preoperative lactate, pH and base excess were evaluated. RESULTS: Differences were noted in preoperative pH (7.28 +/- 0.03 vs. 7.24 +/- 0.01, P = 0.29), base excess (-5.83 +/- 0.64 vs. -6.93 +/- 0.46 mmol/L, P = 0.10) and lactate (3.05 +/- 0.35 vs. 6.08 +/- 0.45 mmol/L, P < 0.001), indicating a significant difference in blood lactate values in favor of the prenatally diagnosed group. CONCLUSIONS: Prenatal diagnosis of congenital heart disease and the resulting immediate postnatal care prevent lactate increase in the preoperative period of these patients. This may decrease the risk of cerebral damage and result in the patient being in better condition at surgery.

Coronary artery fistulas.

The aetiology of congenital coronary artery fistulas remains a challenging issue. Coronary arteries with an anatomically normal origin may, for obscure reasons, terminate abnormally and communicate with different single or multiple cardiac chambers or great vessels. When this occurs, the angiographic morphological appearance may vary greatly from discrete channels to plexiform network of vessels. Coronary arteriovenous fistulas (CAVFs) have neither specific signs nor pathognomonic symptoms; the spectrum of clinical features varies considerably. The clinical presentation of symptomatic cases can include angina pectoris, myocardial infarction, fatigue, dyspnoea, CHF, SBE, ventricular and supraventricular tachyarrhythmias or even sudden cardiac death. CAVFs may, however, be a coincidental finding during diagnostic coronary angiography (CAG). CAG is considered the gold standard for diagnosing and delineating the morphological anatomy and pathway of CAVFs. There are various tailored therapeutic modalities for the wide spectrum of clinical manifestations of CAVFs, including conservative pharmacological strategy, percutaneous transluminal embolisation and surgical ligation.

Right ventricular dysfunction and the role of pulmonary valve replacement after correction of tetralogy of Fallot.

BACKGROUND: Correction of tetralogy of Fallot (ToF) often leads to pulmonary regurgitation, sometimes warranting pulmonary valve replacement (PVR), for which the indications and timing to achieve optimal results are not yet clear. This retrospective study describes follow-up and reinterventions in our ToF population. METHODS: Review of all consecutive patients operated for ToF between 1977 and 2000. Included are date and type of repair, Doppler echocardiography (2D-echo), ECGs, re-operations and physical condition. RESULTS: Total repair was performed in 270 patients, mean age 1.9±2.5 years, 82 were excluded because of follow-up abroad. Right ventriculotomy was used in 92%, transatrial VSD closure in 8%, while 69% received a transannular outflow patch. Pulmonary atresia required a pulmonary graft in 13 (8%) patients. Overall 20-year survival was 88%. Last follow-up: ECG showed RBBB in 67% (QRS complex 129±29.3 msec). RVOT aneurysms were detected in 16%. 2D-echo demonstrated mild pulmonary insufficiency (PI) in 40%, severe in 31%, dilated RV in 76%, both increasing with post-repair age. In 39%, RV dimensions were equal or even exceeded LV dimensions, 45% showed tricuspid insufficiency and the RA was enlarged in 14%. Reintervention was necessary in 39/185 patients, this included angioplasty for residual stenosis and PVR (22/19 homografts, six patients in PA group) at a mean age of 11.2 years after correction. In seven patients, the RV returned to normal dimensions and symptoms disappeared, but in three severe dysfunction developed. Eleven others still have RV dilatation and/or PI. In total, 75% were free of reintervention in the first ten years. The right atrial approach diminishes severe RV dilatation and prolonged QRS duration (p=0.001 and 0.007). Early correction reduces the risk of re-operation (p=0.011). CONCLUSIONS: Severe RV dilatation (39%) and PI (31%) secondary to outflow tract repair in ToF are frequently occurring sequels developing slowly over time. Timing of PVR remains controversial, still best guided by the clinical condition.