Infective Endocarditis in Children as an Increasing Clinical Problem-A Case Series.

In September 2023, the European Society of Cardiology (ESC) published new guidelines for the management of endocarditis. Infective endocarditis (IE) remains a significant life-threatening disease, concerning an increasingly younger age group, especially children with congenital heart disease (CHD) and young adults after multiple cardiac surgeries. This study's aim was to alert the medical community to the problem of increasing IE case numbers and IE course complexity. Of the eight patients who suffered from IE treated in 2023 in our department, we describe the four whose course was the most extraordinary. Afterward, we compared the number of IE patients treated over the years in our department. All cases described children with congenital heart disease suffering from IE. The IE clinical presentation in all patients was extremely diverse, necessitating the utilization of all available diagnostic methods. Each child underwent specialized treatment and subsequently qualified for cardiac surgery. While the etiology and treatment of IE are well established, it remains a formidable challenge for physicians. Pediatric patients who have undergone multiple cardiac surgeries constitute a steadily expanding group and are especially susceptible to IE throughout their lives. Currently, no recommendations exist for the management of endocarditis in pediatric patients. This gap compels pediatricians to adapt existing guidelines designed for adult patients and to rely on scientific reports, such as case studies.

Current practice of care for adolescent and adult patients after Fontan surgery in Poland.

BACKGROUND: The growing number of adult patients after the Fontan operation requires regular surveillance tests in specialized centers. AIMS: Our study aimed to evaluate the current practice of care for Fontan patients in Poland using a multicenter survey. METHODS: Eight centers were included in the study including 5 adult congenital heart disease (ACHD) and 3 pediatric centers for adolescents. To compare the centers and facilitate interpretation of results, the Fontan Surveillance Score (FSS) was developed. The higher score is consistent with better care, with a maximum of 19 points. RESULTS: We included in the study 398 Fontan patients (243 adults and 155 adolescents [aged 14-18 years]). The median FSS was 13 points with variability between centers (interquartile range 7-14 points). Centers providing continuous care from the pediatric period until 18 years of age achieved a higher FSS compared to ACHD centers (median: 14 points vs. 12 points; P <0.001). Most of the patients, both in the ACHD (82.3%) and pediatric centers (89%), were seen annually and had a physical examination, electrocardiogram, and echocardiogram performed at each visit. However, we observed unsatisfactory utilization of tests identifying early stages of Fontan circulation failure (cardiopulmonary exercise tests, cardiac magnetic resonance, liver biochemistry and imaging, detection of protein-losing enteropathy). CONCLUSIONS: Our results showed that there is no unified surveillance approach for Fontan patients in Poland. The practice of care for adults differs from that of adolescents.

A milestone in congenital cardiac surgery: Four decades of the Norwood procedure.

Hypoplastic left heart syndrome (HLHS) was first described by Lev in 1952, but it was not until 1958 that it received a name from Noonan and Nadas. For the next several decades, the defect was considered untreatable. In 1979, William Norwood and his colleagues from Boston initiated a program to evaluate staged surgical management for infants with HLHS. The Norwood operation has became a milestone in the effective palliation for neonates born with HLHS. Today, the Norwood procedure is the first step of a three-stage heart surgery aimed at creating a new circulatory pathway (i.e., the Fontan pathway).

Approaching the 50th anniversary of the first Fontan procedure. What is the current state of treatment provided to patients with functional single ventricles?

Nearly 50 years after the pioneering procedure performed by Francis Fontan and Eugene Baudet, which has saved the lives of thousands of children, there are still more questions than answers regarding therapeutic management. The complex pathophysiology of Fontan circulation, the lack of clear guidelines, and the shift in the care of such patients from pediatric cardiological and cardiac surgical centers to ones dealing with adult patients, cause new threats. This paper outlines the fundamental issues related to the pathophysiology of Fontan circulation and reviews the literature on the methods of treating complications characteristic of this group of patients.

The Two Anomalies of Wilhelm Ebstein.

The year 2016 was the 180th anniversary of Wilhelm Ebstein's birth and the 150th anniversary of his description of the congenital tricuspid valve malformation that came to be known as Ebstein anomaly. We present a brief history of the life and work of Dr. Wilhelm Ebstein. Despite his distinguished career, he is seemingly forgotten in his own birthplace. We include a review of the relevant historical medical literature.

Recanalisation of the left superior caval vein after Fontan procedure: not so rare complication: possibilities of percutaneous closure using various devices.

AIM: Evaluation of possibilities of percutaneous closure of recanalised left superior caval vein after total cavopulmonary connection. METHODS AND RESULTS: We analysed 19 patients after total cavopulmonary connection catheterised because of a sudden increase of desaturation. In four of them, the recanalisation of the left superior caval vein was identified. For this reason, the balloon occlusion tests of the veins were made temporarily. In all cases, the haemodynamic status of patients did not change, and arterial oxygen saturation increased significantly. Thus, using different types of implants, these veins were closed effectively in all patients. During the short-term follow-up, the effectiveness of treatments and constantly maintaining a high level of saturation were confirmed. CONCLUSIONS: Meticulous investigation of unclear causes of desaturation in cyanotic patients after Fontan completion is necessary. Almost all causes of desaturation, including recanalised additional left superior caval vein, can be effectively treated percutaneously.

The radiologist's tragedy, or Bland-White-Garland syndrome (BWGS). On the 80(th) anniversary of the first clinical description of ALCAPA (anomalous left coronary artery from the pulmonary artery).

In 1933, three doctors from the Massachusetts General Hospital in Boston, Paul Dudley White, William Franklin Bland, and Joseph Garland, described a case of an anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA) in a three-month-old boy. The infant died following two weeks of hospitalization. The child's father was Dr. Aubrey Hampton, a radiologist and colleague of White, Bland, and Garland. The paper presents a perspective view on the occasion of the 80(th) anniversary of the first clinical description of ALCAPA.

Development and validation of UHPLC-ESI-MS/MS method for the determination of selected cardiovascular drugs, polyphenols and their metabolites in human urine.

A sensitive ultra-performance liquid chromatography tandem mass spectrometry method with electrospray ionisation (UHPLC-ESI-MS/MS) was developed for the simultaneous determination of 52 compounds: ß-blockers, polyphenols (antioxidants) and their metabolites in mixture of standards and after addition the 52 standard solutions to human urine samples. The analyses of urine samples obtained from patients treated with ß-blockers were also carried out. The separation of analytes was performed on a Hypersil GOLD™ column (100 mm × 2.1mm, 1.9 µm) using a gradient elution profile for 10 min and mobile phase consisting of 0.1% formic acid in water and acetonitrile. In these conditions, some of the tested compounds were not separated, but this was compensated by the use of MS/MS detection. The drugs, polyphenols and their metabolites were detected with a tandem mass spectrometer after being ionised positively or negatively (depending on the molecule) using an electrospray ionisation (ESI) source. The MS system was operated in the selected reaction monitoring (SRM) mode, where one quantitation and one confirmation transition was done for each analyte. The quantitative method was validated for selectivity, linearity, low limits of quantitation, accuracy, precision, recovery, matrix effect and analyte stability. The LLOQ varied from 0.01 to 0.40 ng mL(-1) for ß-blockers and from 0.05 to 40.0 ng mL(-1) for polyphenols. The linear range was 0.08-1000 ng mL(-1) for the drugs and 0.10-2300 ng mL(-1) for the polyphenols. Intra-day and inter-day precision was less than 8%, and the accuracy ranged from -4.40 to 2.23% for all analytes. The average recoveries for all compounds analysed were better than 90%. The developed method can be successfully used to monitor cardiovascular drugs and their metabolites in urine samples of patients treated with ß-blockers and can also be used to study the effect of polyphenols on the metabolism of drugs.

Transcatheter closure of ruptured sinus of valsalva aneurysms with an Amplatzer occluder.

We report the transcatheter closure of six ruptured sinus of Valsalva aneurysms (RSVAs) in five patients aged 18-51 years. The RSVA extended into the right atrium in three patients, into the right ventricular outflow tract in one, and into the pulmonary artery in one. In all patients, the RSVA was entered from the aorta, an arteriovenous loop was created, and the Amplatzer occluders were implanted using a venous approach. Six procedures were performed in five patients. Five Amplatzer duct occluders and one Amplatzer atrial septal occluder were implanted. In one patient who had a left RSVA after deployment of an Amplatzer duct occluder, ST-segment depression was observed on the ECG and the procedure was abandoned. In the other four patients, complete closure of the RSVA was confirmed by color Doppler echocardiography. No complications were observed during 9-19 months of follow-up. Percutaneous closure of an RSVA is feasible and can be used as an alternative to surgery.

Closure of atrial septal defect with the Amplatzer septal occluder in adults.

Transcatheter closure of atrial septal defects (ASDs) was performed mainly in children and adolescents. Information about outcome and complications in adults was limited. From November 1997 to November 2005, percutaneous closure of ASDs using the Amplatzer septal occluder was attempted in 650 consecutive adult patients. Median patient age was 45.8 +/- 16.2 years (range 18 to 90), mean systolic artery pressure was 33.3 +/- 10.6 mm Hg (range 11 to 85), and mean pulmonary and systemic blood flow (Qp/Qs) ratio was 1.9 +/- 0.7 (range 0.8 to 6.6). Mean stretched diameter of the ASD was 21.2 +/- 5.1 mm (range 3.1 to 43). Seventy-eight patients (12%) had multiple defects. Of 572 patients with a single ASD, device implantation was successful in 563 patients (98%). During follow-up, complete closure could be achieved in 96% of patients with a single ASD and 71% of patients with multiple defects. Mean systolic artery pressure decreased to 28.3 +/- 10.1 mm Hg and mean Qp/Qs ratio decreased to 1 +/- 0.3. The 3 complications that occurred during the procedure were device embolization (2 patients; 0.3%) and transient ST depression (1 patient; 0.2%). The most common complication immediately after the procedure and during follow-up was new-onset atrial fibrillation (28 patients; 4.3%). Electrical cardioversion was successfully in most. Complications requiring emergency or elective surgery occurred in 6 patients (0.9%; hemopericardium, 2 patients, 0.3%; device embolization, 3 patients, 0.5%, and pericardial tamponade, 1 patient, 0.2%). In conclusion, closure of ASDs using the Amplatzer septal occluder in adults was efficient and safe, with excellent long-term success rates. Serious complications were rare.

Utility of the dobutamine stress echocardiography in the evaluation of the effects of a surgical repair of aortic coarctation in children.

BACKGROUND: Exercise-induced hypertension following repair of the coarctation of the aorta (CoA) is a well known phenomenon. The most important functional parameters in the assessment of the effects of a surgical repair of CoA are the maximal pressure gradient in the descending aorta (GRAD) and systolic blood pressure (SBP). Results of treadmill exercise test using the Bruce protocol (treadmill test) and dobutamine stress echocardiography (DSE) were compared to determine utility of the DSE in the evaluation of the effects of surgical treatment of CoA in children. METHODS: The study population comprised of 29 patients, including 20 males and 9 females (mean age 12 years) who underwent a surgical repair of CoA. Changes of the cardiovascular parameters including SBP, GRAD and heart rate (HR) during the treadmill test and DSE were compared. RESULTS: During the treadmill test, SBP at peak exercise ranged from 120 to 230 (mean 163.7) mm Hg, GRAD ranged from 29 to 109 (mean 59.8) mm Hg, and HR ranged from 140 to 188 (mean 169) bpm. At the end of DSE, SBP ranged from 123 to 215 (mean 164.7) mm Hg, GRAD ranged from 29 to 113 (mean 55.4) mm Hg, and HR ranged from 76 to 155 (mean 111) bpm. We found positive correlations of SBP (r = 0.68, p < 0.001) and GRAD (r = 0.82, p < 0.001) values during both tests but no significant correlation for HR (r = 0.42, p = NS). CONCLUSIONS: Dobutamine stress echocardiography is useful in the evaluation of the effects of surgical repair of CoA in children.

The use of two Amplatzer "Cribriform" Septal Occluders to close multiple postinfarction ventricular septal defects.

Rupture of the interventricular septum is an uncommon and often fatal complication of myocardial infarction. Herein, we report the successful deployment of the Amplatzer Multi-Fenestrated Septal Occluder-"Cribriform" (AGA Medical Corporation; Plymouth, Minn) during 2 procedures to close multiple postinfarction ventricular septal defects in a severely ill patient. We show that, in selected patients who have multi-fenestrated multiple postinfarction ventricular septal defects, transcatheter implantation of the Amplatzer Cribriform Occluder can be a good therapeutic option.

[Transcatheter closure of postinfarction ventricular septal defects using Amplatzer devices]. / Cierre percutáneo de comunicaciones interventriculares postinfarto mediante los dispositivos de Amplatzer.

We carried out transcatheter procedures to close postinfarction ventricular septal defects (PIVSDs) in 19 patients: two had recanalization after surgical closure, and 17 had a primary PIVSD. In three of the latter patients, who had acute PIVSDs, the procedure was carried out in the first 3 weeks after infarction; in the 13 patients with subacute PIVSD, it was carried out 3.5-12 weeks after infarction. There was another procedure in one patient with chronic PIVSD. In total, 22 procedures were completed: 17 using an Amplatzer atrial septal occluder, two using an Amplatzer postinfarction ventricular septal defect occluder, and two using an Amplatzer muscular ventricular septal defect occluder. The procedure was successful in 14 patients: in 11 with subacute PIVSD, one with chronic PIVSD, and two with postsurgical PIVSD. Transcatheter closure of PIVSDs using an Amplatzer atrial septal occluder is probably the treatment of choice in patients undergoing surgery more than 3.5 weeks after myocardial infarction and in those with recanalization after previous surgical closure.