To assess the effect of a modified Fontan operation on systemic venous blood flow and the hepatic circulation, we compared 11 patients having an atriopulmonary connection and 35 with total cavopulmonary anastomosis. The Doppler echocardiographic study of the caval venous, hepatic venous and portal venous flow was performed so as to calculate the pulsatility ratio and the variation of flow with respiration. All patients had undergone cardiac catheterization. In addition, we included specific laboratory investigations to assess function of various organs.

Significantly lower maximum velocities of flow at inspiration (0.31±0.12 rn/sec vs 0.45±0.14 m/sec) and expiration (0.23±0.09 rn/sec vs 0.32±0.11 m/sec), less pulsatility (0.43 vs 0.16) and a lower ratio of systolic to diastolic velocity (1.22 vs 1.85) were found in the patients having a cavopulmonary as compared to an atriopulmonary anastomosis. Peak velocities of hepatic venous flow during inspiration and expiration were significantly lower in those with a cavopulmonary anastornosis (p=O.OOl and p<O.OOl, respectively). In these patients, forward flow was extremely dependent on respiration, with decrease or cessation of antegrade flow during expiration in 22 patients. The velocity of portal venous flow was also significantly lower in these patients, although the pulsatility ratio did not differ significantly between the groups (0.5 ± 0.21 and 0.57 ± 0.23, respectively). The ratio of inspiratory and expiratory velocities showed no significant difference between the groups, nor was there any correlation between the pulsatility ratio of the venous vessels or the ratio of peak flow velocities during expiration and the mean systemic venous/right atrial pressure on postoperative cardiac catheterization. Hypoproteinemia was found in 8 patients after total cavopulmonary anastomosis; 9 of 10 patients with protein C deficiency belonged to this group.

The dependence of hepatic venous flow on respiration in the presence of a chronically elevated systemic venous pressure in patients after the total cavopulmonary anastornosis may influence hepatic function in the postoperative period.

A 17-year-old adolescent with severe multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease-2019 developed reduced left ventricular function and left ventricular thrombus. With treatment, his condition improved and the thrombus was dissolved. This case illustrates the risk of severe intra-cardiac thrombotic complications in patients with MIS-C.

We present two cases of percutaneous Sapien XT valve-in-valve implantation in the tricuspid position: a 20-year-old man with severe congenital pulmonary stenosis and percutaneous valvuloplasty, who required surgical implantation of two protheses, pulmonary and tricuspid, and a 12-year-old boy with CHD and a degenerated tricuspid prosthesis. We implanted three Sapien XT valve-in-valves, two in the tricuspid position and one in the pulmonic position. Sapien XT valve-in-valve implantation in the tricuspid position is feasible and can decrease the number of surgeries in CHD patients.

Although uncertainty exists, in some situations, as to the optimal type of operation and a true cure may not always be possible, the outlook for patients with all types of complete transposition is poor without surgical treatment. With surgery, and in spite of current uncertainties as to the optimal type of repair, most patients can be greatly benefited, and many given a long and happy life. Even more should be possible in the future.

We report on the management of Candida endocarditis in a 5-month old infant. The orifice of the tricuspid valve was totally obstructed, and the tension apparatus of the valve destroyed. Excision of the valve led to severe failure of the right heart. The combined use of anti-failure treatment, and reduction of right ventricular afterload with oxygen, nitric oxide and sildenafil, proved successful.

Cardiac rhythm monitoring has been facilitated by the use of implantable loop recorders. New models of these devices are 87% smaller than before allowing for easier implantation and use in the paediatric population. Recommendations are for closure with adhesive. We report a device extrusion in a 6-year-old patient. Based on this, our practice has changed to include subcutaneous sutures this complication.

The size of the coronary arteries parallels the ventricular mass, thus it may be abnormal in complete transposition beyond infancy and could be influenced by the arterial switch operation. To investigate this possibility, we measured the diameters of the right, left main trunk, anterior descending, and circumflex coronary arteries before and three to seven years (mean 4.8) after the arterial switch operation in 17 patients with a “normal” distribution of the coronary arteries (so-called Shaher type 1). The values were compared with 18 controls who had Kawasaki disease with no apparent coronary arterial disease. The right, left anterior descending, and circumflex arteries were smaller than control values before the operation. The post-/preoperative ratios of the diameter were 1.16±0.11 for the right coronary artery, 1.18±0.16 for the left main trunk, 1.20±0.18 for the left anterior descending artery, and 1.22±0.26 for the circumflex artery. There were no significant differences among these values. After surgery, the right coronary artery was larger, but the left coronary arteries were smaller in the patients than in the controls: 2.5±0.3 vs 2.0±0.2 mm for the right coronary artery; 2.4±0.3 vs 2.7±0.1 mm for the left main trunk; 1.9±0.2 vs 2.4±0.2 mm for the left anterior descending artery; 1.6±0.4 vs 2.2±0.5 mm for the circumflex artery, respectively. The posterior descending coronary artery originated from the right coronary artery in all patients. The total cross-sectional area of the right coronary, left anterior descending, and circumflex arteries was 9.7±2.4 mm2 in the patients, and 11.8±2.9 mm2 in the controls (p>O. 1), suggesting that the increased size of the right coronary artery compensates for the small left coronary arteries. We conclude that the arterial system in complete transposition, with a large right coronary artery and small left coronary system, remains smaller than normal even at midterm follow-up after anatomic repair despite normalization of left ventricular volume and muscle mass.

The reentrant circuit of common atrial flutter is known to located in the right atrium between two anatomical barriers. Recent electrophysiologic studies have defined the tricuspid annulus as the anterior barrier, and the terminal crest and its continuation as the eustachian ridge as the posterior barrier. Construction of a bidirectional block to conduction between these two barriers by means of lesions created with radiofrequency current have been shown to be effective in ablating the flutter. We now find that surgical creation of such a block to conduction between the barriers by a simple modification of the atrial incision line is equally effective. In a 6-year-old boy, who was admitted to our hospital for closure of an atrial septal defect and treatment of sustained atrial flutter, the atriotomy was performed perpendicular to the terminal groove and extended towards the tricuspid annulus, placing some crythermal lesions between the end of the incision and the annuals. The special defect was closed using a Dacron patch. The child was free of arrhythmia both during the postoperative stay and over the initial three months of follow-up. We conclude that this simple modification of the atrial incision line provides cure of atrial flutter in children who require atriotomy for repair of congential cardiac anomalies. It may also be beneficial in preventing ‘incisional’ reentrant tachycardia.

Two neonates with severe pulmonary stenosis deteriorated immediately after successful balloon valvoplasty as a result of increased infundibular obstruction. They were treated with β-adrenoceptor blockers and intravascular expansion with limited success. Phentolamine was then given, resulting in dramatic improvement. The children could be weaned from the ventilator within hours of starting this new therapeutic modality.

Background: The aim of this study was to evaluate our experience in central extracorporeal life support with an integrated left ventricular vent in children with cardiac failure. Methods: Eight children acquired extracorporeal life support with a left ventricular vent, either after cardiac surgery (n = 4) or during an acute cardiac illness (n = 4). The ascending aorta and right atrium were cannulated. The left ventricular vent was inserted through the right superior pulmonary vein and connected to the venous line on the extracorporeal life support such that active left heart decompression was achieved. Results: No patient died while on support, seven patients were successfully weaned from it and one patient was transitioned to a biventricular assist device. The median length of support was 6 days (range 5–10 days). One patient died while in the hospital, despite successful weaning from extracorporeal life support. No intra-cardiac thrombus or embolic stroke was observed. No patient developed relevant intracranial bleeding resulting in neurological dysfunction during and after extracorporeal life support. Conclusions: In case of a low cardiac output and an insufficient inter-atrial shunt, additional left ventricular decompression via a vent could help avoid left heart distension and might promote myocardial recovery. In pulmonary dysfunction, separate blood gas analyses from the venous cannula and the left ventricular vent help detect possible coronary hypoxia when the left ventricle begins to recover. We recommend the use of central extracorporeal life support with an integrated left ventricular vent in children with intractable cardiac failure.

Distinguishing the benign and malignant cardiac masses is usually a diagnostic challenge, and safe, non-invasive, and reliable techniques are necessary to avoid any delay in treatment. The previously described “sun ray” appearance is an important sign in the diagnosis of cardiac angiosarcoma, and in this report we present 3-T magnetic resonance imaging findings to distinguish the rare but fatal cardiac angiosarcoma.

A retrospective analysis of 55 patients who underwent replacement of the left atrioventricular valve following surgery for atrioventricular septal defect with separate right and left atrioventricular valves or with a common valvar orifice has revealed a marked decrease in the need for valvar replacement over time. Between 1983 and 1987, the incidence of replacement following repair has been 3% in infants. Overall early mortality for the entire series, extending from 1970 to 1987, was 22%. Mortality in the first year of life was 58%, which was significantly greater than mortality beyond one year of age (14%, p<0.01). Obstruction of the left ventricular outflow tract was a frequent incremental risk factor for death during infancy. The incidence of complete heart block (25%) is disappointingly high, and may reflect distortion of the left atrioventricular junction in an attempt to place as large a prosthesis as possible. The incidence of complete heart block has not decreased with time. These results confirm the desirability of secondary valvoplasty procedures to enable a child to be larger before ultimate replacement of the valve is required.

Background: Elevation of cardiac troponin I in the serum is a specific marker for myocardial injury. We measured levels of troponin I in the serum in children before and after cardiac catheterization to determine if this procedure was associated with an increase in levels of troponin. Methods: We enrolled patients under 21 years of age undergoing cardiac catheterization at our institution. A baseline sample of serum was drawn at the start of the procedure. Repeat samples were obtained immediately after, and six hours subsequent to the procedure. All samples were analyzed for cardiac troponin I using the Abbott AxSYM microparticle immunoassay system. Levels were considered normal (0–0.4 ng/ml) or elevated (>0.4ng/ml). Patients were excluded if the baseline level was elevated. Results: Levels of cardiac troponin I were elevated in the serum from 11 of 14 (79%) cases immediately after the procedure (p < 0.0001), and in 12 of 14 (86%) six hours later (p < 0.0001). Only 2 patients had recognized complications potentially causing myocardial injury. Conclusion: Levels of cardiac troponin I increase in the serum in a high proportion of children after cardiac catheterization. These elevations can be observed immediately, and are maintained for at least six hours. Our study suggests that cardiac catheterization, predominantly intervention, is associated with myocardial injury, even in the absence of complications.