CHD may, at times, occur in the framework of other rare pathologies. These, having similar clinical manifestations, present a diagnostic dilemma for the clinician.

The authors present the case of an infant with non-syndromic complete atrioventricular septal defect, whose post-operative period was surprisingly complicated by progressive pulmonary hypertension. Despite intensive care, the infant ultimately died. The diagnosis of unilateral primary pulmonary lymphangiectasia was only possible post mortem.

Undiagnosed coronary sinus obstruction can be a source of paediatric exertional chest pain and death. We present a case series of three interesting children who developed only exertional angina from various degrees of coronary sinus stenosis or atresia. We discuss the diagnosis and management of each case and the potential pathophysiology involved with their symptoms.

One of the important complications of transcatheter occlusion of the patent duct by insertion of either the Rashkind double umbrella or coil devices is intravascular haemolysis, particularly the severe form which occurs in 0.5–0.6% of cases. The incidence of subclinical or mild intravascular haemolysis including morphologic changes in the red cells, however, is not known, especially in the group of patients with residual shunt. We studied laboratory and haematologic changes in 37 patients subsequent to transcatheter occlusion. Their ages ranged from 2 years 2 months to 11 years 8 months (6.2 + 2.7 years), and the duration of occlusion had ranged from 1 month to 2 years 11 months (1.24 + 0.89 years). In 30 cases we had used the Rashkind double umbrella device, and nine of these patients (30%) had a residual shunt. The other seven ducts were closed with detachable coils, and five of these cases (71%) had residual shunting. There was evidence of mild or subclinical intravascular haemolysis in almost half the cases, although no patient developed acute intravascular haemolysis. Fragmented red cells, blur cells and spherocytes from blood smears were found in up to one-third of the overall series, with no differences found according to persistence of shunting. Haemosiderin was found in the urine of the group with residual shunting and in 13–23% of those with complete occlusion. Serum hypohaptoglobin was found in 9–13% of the patients with a residual shunt, and in 9–23% of those with complete occlusion. Four of the patients had haptoglo-bin levels <50 mg/dl. There were no patients with reticulocytes counts >2% in the blood smears. We conclude that subclinical or mild intravascular haemolysis, along with morphologic changes in the red blood cells, are significant findings subsequent to occlusion of the patent arterial duct with either the Rashkind double umbrella or detachable coils.

Transesophageal echocardiography and magnetic resonance imaging have proved useful as noninvasive imaging modalities in postoperative patients with congenital heart disease, or patients with suboptimal transthoracic echocardiograms. Each modality has specific advantages. When both are of equal efficacy, magnetic resonance imaging is recommended because it is less invasive and more comfortable for the patient.

Our purpose was to study serially, measurements which relate to right ventricular events, using echocardiography and Doppler in full term neonates for the first three weeks after birth. Echocardiographic and pulsed Doppler studies were performed for 21 newborns during the first 36 hours of life, and subsequent serial studies were performed in the second (n=21) and third (n=14) weeks of life. Evaluation included measurement of right isovolumic relaxation time and peak E and A velocities across the tricuspid valve. Pulmonary velocities were studied for evidence of patency of the arterial duct, and to determine acceleration in the pulmonary trunk. Mean right isovolumic relaxation time decreased significantly from 68±9 ms to 39 ± 14 ms (p<0.001) between the first and second week. Mean tricuspid peak E increased significantly between the second (48 ± 7 cm/s) and the third week of life (62 ± 8 cm/s; p<0.001). Mean peak A wave velocity did not change significantly during the time of study. The pulmonary arterial acceleration time increased from the initial measurement (68 ± 10 ms) to the second week (85 ± 9 ms; p<0.001). Changes in right isovolumic relaxation time were significantly related to the changes in the pulmonary arterial acceleration time (r=0.63, p=0.0001), suggesting a significant influence of afterload on diastolic events. These data demonstrate the normal progression of some measures of early right ventricular diastolic function.

Formation of aortic aneurysms is an important and not infrequent sequel of surgical repair of aortic coarctation. Detection is often haphazard and fatal ruptures are reported. We report a series of cases where resonance imaging identified aneurysmal dilatation of the thoracic aorta after either patch aortoplasry or subclavian flap repair of aortic coarctation. In one, rupture of the aneurysm occurred five months following the initial resonance study. Repeat imaging confirmed the clinical suspicion of rupture and successful surgical resection was subsequently performed. Magnetic resonance is a practical and reproducible imaging technique which is particularly valuable in the identification of abnormalities within the thoracic aorta following surgical aortoplasty or interventional procedures for aortic coarctation. As a method of routine surveillance in these groups of patients, it is superior to any alternative technique.

From January 1988 through to July 1994, 54 consecutive infants underwent an arterial switch operation for simple or more complex forms of complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections). They ranged in age from 2 to 180 days. The Lecompte maneuver was performed in all. In the first 19 patients the harvested sinuses of Valsalva were filled with two separate patches of autologous preserved pericardium, while, in the last 35 patients, a wide pantaloon patch of tanned pericardium was employed. Five babies died within 30 days after the procedure (operative mortality of 9.2%, 70% CL 4–17%). Four survivors were found to have developed a significant supravalvar pulmonary stenosis from 1 month to 21 months postoperatively. Echocardiographic data showed a transpulmonary peak systolic gradient from 60 mmHg to 101 mmHg, with a right-to-left ventricular systolic pressure ratio from 0.65 to 0.9. Reoperation was performed from 8 months to 39 months after the arterial switch procedure. Supravalvar pulmonary stenosis was located at the level of the pulmonary trunk, extending distally in two cases, due to the growth of fibrous scarring tissue with partial calcification. The pulmonary valvar leaflets were involved in two cases. Relief of the obstruction was obtained by insertion of a wide shield-tailored polytetrafluoroethylene patch after making an inverted Y-shaped longitudinal incision in the pulmonary trunk between the anterior sinuses of Valsalva. No mortality occurred at reoperation. Early and midterm echocardiographic measurements showed the effectiveness of this technique, with only trivial or mild residual transpulmonary gradients.

This case report presents an infant patient with the association of trisomy 7p and tetralogy of fallot (TEF). Patients diagnosed with trisomy 7p should certainly be scheduled for an echocardiographic exam and be scanned for any CHDs that may accompany it. The CHDs that most frequently accompany this syndrome include atrial septal defect, ventricular septal defect, and patent ductus arteriosis. Yet, it should be known that TEF may also be present, albeit rarely.

Pregnancy in a woman with double outlet right ventricle, ventricular septal defect, and pulmonary stenosis is presented. Her previous two pregnancies ended in fetal losses. In the current pregnancy, she had thrombocytopenia, placenta previa, and severe fetal growth restriction. She underwent an emergency caesarean section followed by post-partum haemorrhage and COVID-19 infection. Literature review has been performed.

The role of the pathologist is changing with regard to the assessment of postoperative congenital heart disease. Whereas, in the past, the access to autopsied hearts provided the opportunity for detailed description and classification, nosologic details of cardiac malformations are now largely agreed. While a detailed analysis of the lesion remains an integral part of the pathologist's task, future advances will now largely be dictated by careful analysis of the effects of cardiac surgery. Such analysis can be divided into early and late phases. When assessing the specimen from a death occurring in the operating room or in the immediate postoperative period, the pathologist must examine carefully the operative incisions, and the correctness of the surgical procedure. Proper assessment of the procedure will necessitate removal of patches and pathways constructed by the surgeon, having documented carefully the location of sutures used to secure the foreign materials. The autopsy must also include assessment of the organs in the rest of the body, particularly the lungs, and must not ignore the immediate effect of the operative procedure on myocardial status. In the late phase, while again assessing in detail the access to the heart and the procedures employed during surgical correction, the pathologist will take care to evaluate the long-term effects of the procedure on the valves of the heart, the myocardium, the pulmonary vasculature and the other organs. At this stage, care should also be taken to ensure that late scarring does not involve the conduction tissues. Particular care will be needed when assessing the efficacy and durability of prosthetic materials inserted into the heart. Most of these steps will be undertaken intuitively by the pathologist, but interest in cardiac pathology as a specialty seems to be waning. The clinician can combat this trend by ensuring that all cases dying after operative procedures are obtained for autopsy, and by encouraging the pathologist to become involved in this fascinating area.

Between 1968 and December 1990, 29 patients underwent 33 reoperations for dysfunction of the left atrioventricular valve after correction ofatrioventricular septal defect. Repair of the valve was possible in all 10 patients who, initially, had a common atrioventricular orifice, using straightforward procedures such as closure of the septal commissure and annuloplasties according to Wooler. Additional valvar abnormalities (double orifice; fenestration of leaflets; malpositioned or malformed papillary muscles; and additional clefts) were rare in this group of patients. In contrast, these anomalies were frequent in the 19 patients who, initially, had separate right and left atrioventricular valves.

Absence of myocardial fibers in the right ventricle is the essence of so-called Uhl's anomaly, which should be distinguished from the fatty replacement producing arrhythmogenic right ventricular dysplasia of the adolescent and young adult. In this report, we describe a newborn with nearly complete absence of the myocardium of the left ventricle. The infant died on the seventh day because of myocardial incompetence of the left ventricle, which was unable to open the aortic valve.

In western Sweden, less than one-third of children born with complete transposition between 1964 and 1983 are long-time survivors. During bicycle ergometry, using a steady-state protocol with analyses of the expiratory gases and lactate, we investigated the exercise capacity in survivors of atrial redirection. There were 16 patients who had a Mustard procedure, now aged 16.9±3.6 years (12.0–22.0) and 15 patients who underwent a Senning correction aged 9.4±1.6 years (7.2–12.1). Nineteen percent of Mustard cases and 53% of Senning cases had minor or no sequels. Moderate sequels were found in 44% of post-Mustard cases, and 40% of post-Senning cases, including baffle complications, moderate systemic ventricular dysfunction, sinus node dysfunction and tracheal stenosis. Severe problems were encountered in 37% of post-Mustard cases and 7% of post-Senning cases, including pulmonary hypertension, pulmonary venous obstruction, atrioventricular block and hemiparesis. The maximal exercise capacity was low (p<0.0001); 71% had an oxygen uptake below −2 SD of predicted values, with severely impaired oxygen uptake in all except one of the cases with severe sequels, but no significant difference was found between patients having mild and moderate sequels. The maximally achieved heart rate was low (p<0.0001); 71% had values below −2 SD of predicted, including all cases with sinus nodal dysfunction, as well as 40% of cases without it. The maximal oxygen pulse was low (p<0.01), below −2 SD in 36%, but it was less affected than other exercise variables in 68% of the patients. Our study, therefore, presents exercise data in an epidemiologically-based population, relates the data to postoperative sequels and discusses some possible adaptive mechanisms.

We describe a case of Candida endomyocarditis in a neonate not diagnosed until autopsy. Echocardiography had demonstrated a left atrial mass. Since cardiac candidiasis seems to be occuring with greater frequency in neonatal intensive care units, echocardiography and blood cultures should be done to exclude this previously rare event.

Sudden death in pulmonary atresia and intact ventricular septum with right ventricular dependent coronary circulation is a well-established complication, and is thought to be caused by myocardial ischaemia. We report a case of paroxysmal complete atrioventricular block that raises the possibility of an additional mechanism of sudden death.

Ductus arteriosus is a physiological structure if not closed after birth, may lead to many complications. Today, trans-catheter closure of patent ductus arteriosus with Occluder devices is the preferred method. Surgical ligation is used only in certain cases such as large symptomatic patent ductus arteriosus in very small infants and premature babies; unfavourable structure of the duct or economic considerations. In this article, we described haemodynamic and morphological characteristics of five patients with large patent ductus arteriosus which were occluded with Amplatzer device.

From 23 January, 2010 to 31 July, 2018, five patients referred to our clinic with large patent ductus arteriosus and pulmonary arterial hypertension for further evaluation. After assessing them with various diagnostic methods, we decided to close defect with ventricular septal defect Occluder device. Patients aged 21–44 years and one of them was male. Ductus closure was successfully done with ventricular septal defect Occluder device. Closure was successful for all of them but in one case, whose device was embolized to pulmonary artery after 24 hr and he underwent surgery.

Trans-catheter closure of large patent ductus arteriosus in adult patients with pulmonary hypertension is feasible. Despite the fact that complications may occur even with the most experienced hands, the ‘double disk’ Amplatzer ventricular septal defect muscular Occluder could be advantageous in this setting.

A 4-year-old boy presented to our institution with pancytopenia, consumptive coagulopathy, hepatosplenomegaly and recurrent complex pericardial effusion secondary to kaposiform lymphagiomatosis. Due to extensive loculation, conventional drainage was minimally effective. As an adjunct to medical therapy, the Indigo™ aspiration system was used to remove thrombus within the pericardial space. Our patient had good medium-term results with complete resolution of his pericardial effusion at 4 months.

Plasma leakage in polytetrafluoroethylene grafts arises through complex interactions between the prosthetic physical/chemical microstructure and patient environment. While many of the precipitating factors can be avoided by careful graft-handling and meticulous surgical technique, this unpredictable complication still contributes significant morbidity to shunt procedures. Protein sealing of the graft offers a possible solution to the problem.