A Wide Comparison of Techniques for Repair of PAPVCs: One Institution's 20-Year Experience.

BACKGROUND: Different methods for surgical correction of partial anomalous pulmonary venous connection (PAPVC) exist. We evaluated the outcomes of four techniques regarding morbidity and mortality. METHODS: A total of 116 patients underwent PAPVC repair in our institution over a period of 20 years. Single-patch technique (n = 82 [71%], mean age: 18.59 ± 20.49 years), double-patch technique (n = 13 [11%], mean age: 43.18 ± 25.14 years), Warden's technique (n = 7 [6%], mean age: 10.04 ± 10.47 years), and direct implantation of anomalous pulmonary veins (n = 14 [12%], mean age: 14.42 ± 18.58 years) were examined. RESULTS: Out of the 116 patients, one patient (0.9%) developed pulmonary hypertension after discharge and three patients (2.6%) with normal right cardiac function showed right ventricular failure. In total, a pacemaker was inserted in seven cases (6%). Three patients (2.6%) presented with persistent nonsinus rhythm during follow-up. This complication was most frequently seen in the double-patch group being significantly increased compared with the other groups (p = 0.035). One patient presented with a mild stenosis of the superior vena cava. There were two early, nonsurgery-related deaths and no late mortality. CONCLUSION: Operative therapy of PAPVC has low postoperative morbidity and mortality. Therefore, surgical repair of this cardiac anomaly is a safe and reproducible treatment independent of the applied method. The surgical technique must be selected based on the anatomy and possible accompanying congenital heart defects. Special care should be taken when using the double-patch technique because of significant more frequent nonsinus rhythm events postoperatively.

[Linear scleroderma "en coup de sabre" causing severe neurological and ocular symptoms]. / Lineare Sklerodermie "en coup de sabre" mit neurologischer und okulärer Beteiligung.

We present a rare case of a 4-year-old girl suffering from a sclerotic lesion on the forehead as well as uveitis and epileptic seizures. The patient was diagnosed with linear scleroderma "en coup de sabre" (LSECDS). Faced with no gold standard for treatment of LSECDS with severe extracutaneous manifestations, the case was intensively discussed within an interdisciplinary team setting and successfully treated.

Cor triatriatum sinister with left anomalous pulmonary venous return to innominate vein.

Cor triatriatum sinister is a rare congenital abnormality, with varying signs and symptoms related to the anatomic features and the components of the accompanying cardiac abnormalities. We present a 3-year-old girl who underwent successful simultaneous operation with complete loss of the membrane and ligation of vertical vein by anomalous venous return. The principles for surgical management of cor triatriatum can be summarised as radical resection of the membrane and correction of other intracardiac anomalies.

Cyclooxygenase-2 expression in lung in patients with congenital heart malformations and pulmonary arterial hypertension.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a cause of morbidity in patients with congenital heart disease (CHD). It has been hypothesized that prostanoides participate in the development of PAH. The aim of this study was to show the potential expression of cyclooxygenase-2 (COX-2) in patients with CHD and PAH. PATIENTS AND METHODS: We included patients with isolated left-to-right shunts undergoing lung biopsy before or concomitantly with cardiac surgery between 2004 and 2009.For determination of COX-2 expression, histological and immunohistochemistry analyses as well as quantitative polymerase chain reaction (qPCR) were performed. RESULTS: We were able to show COX-2 protein overexpression in the lung tissue of children with CHD and PAH. Furthermore, we showed an increase in COX-1 gene expression and an even stronger induction of COX-2 by using qPCR and immunohistochemistry. CONCLUSIONS: We examined the expression of COX-2 in lung tissue from patients with CHD and PAH. We showed that COX-2 is expressed in diseased lung tissue, indicating a relationship between COX-2 and vascular remodeling in pulmonary arteries in CHD.

Surgical Treatment Following Stent Angioplasty for High-Risk Neonates With Critical Coarctation of the Aorta.

BACKGROUND: Neonatal coarctation of the aorta (CoA) is primarily treated by surgical repair. However, under certain high-risk constellations, initial stent angioplasty may be considered followed by surgical repair. We report our experience with this staged approach. Methods: All patients undergoing surgical CoA repair following prior stenting at our institution between January 2011 and December 2019 were included in this retrospective analysis. The patients were classified to be at high risk because of cardiogenic shock, associated complex cardiac malformations, neonatal infection, necrotizing enterocolitis, and extracardiac conditions, respectively. Outcomes were analyzed and compared with neonates who underwent surgical CoA repair without prior stenting in the same observation period. RESULTS: Twenty-six neonates received stent implantation at a median age of 20 days (IQR 9-33 days). Subsequent surgical repair was conducted at an age of 4.2 months (IQR 3.2-6.1 months) with a median body weight of 5.6 kg (IQR 4.5-6.5 kg). Cardiopulmonary bypass was applied in 96% of cases. Extended end-to-end anastomosis was possible in 11 patients. Extended reconstruction with patch material was necessary in the remaining patients. One fatality (3.8%) occurred 33 days postoperatively. At a median follow-up of 5.2 years after initial stenting, all remaining patients were alive; 15/25 patients (60%) were free from re-intervention. Of note, re-intervention rates were comparable in neonates (n = 76) who were operated on with native CoA (28/74 patients; 38%; P = .67). CONCLUSIONS: Neonatal stent angioplasty for CoA results in increased complexity of the subsequent surgical repair. Nevertheless, this staged approach allows to bridge high-risk neonates to later surgical repair with reduced perioperative risk and acceptable midterm outcomes.

Psychosocial Impact of Congenital Heart Diseases on Patients and Their Families: A Parent's Perspective.

BACKGROUND: Currently, over 90% of children with congenital heart disease (CHD) survive into adulthood. As a consequence the psychosocial impact on children and their families has become an important outcome measure. Therefore, the goal of this study was to assess the psychosocial impact from a parent's perspective and to identify possible predictors. METHODS: We included all parents of children who underwent open-heart surgery in the years 2010 and 2011 at the Department of Cardiothoracic Surgery at University Hospital Heidelberg and invited them to complete standardized questionnaires. Psychosocial outcome was measured via parent self- and proxy reporting of family burden (Family Burden Questionnaire, FaBel), health-related quality of life (KidScreen-10), developmental problems (Five-to-Fifteen, FTF), and mental health problems (Strength and Difficulties Questionnaire, SDQ). RESULTS: In total, 113 families returned the questionnaires completely (71.5%). The Aristotle Basic Complexity score and the STAT 2020 Score overall did not predict the psychosocial impact, whereas the number of surgical operations did significantly predict psychosocial impact across all domains in this study cohort. CONCLUSIONS: These data suggest that the number of surgical operations might be a relevant predictor for the long-term psychosocial impact on families suffering from CHD and a potential connecting factor for specialized psychological support. When setting up screening instruments or support programs the entire family must be considered.

DYRK1A is a novel negative regulator of cardiomyocyte hypertrophy.

Activation of the phosphatase calcineurin and its downstream targets, transcription factors of the NFAT family, results in cardiomyocyte hypertrophy. Recently, it has been shown that the dual specificity tyrosine (Y) phosphorylation-regulated kinase 1A (DYRK1A) is able to antagonize calcineurin signaling by directly phosphorylating NFATs. We thus hypothesized that DYRK1A might modulate the hypertrophic response of cardiomyocytes. In a model of phenylephrine-induced hypertrophy, adenovirus-mediated overexpression of DYKR1A completely abrogated the hypertrophic response and significantly reduced the expression of the natriuretic peptides ANF and BNP. Furthermore, DYRK1A blunted cardiomyocyte hypertrophy induced by overexpression of constitutively active calcineurin and attenuated the induction of the hypertrophic gene program. Conversely, knockdown of DYRK1A, utilizing adenoviruses encoding for a specific synthetic miRNA, resulted in an increase in cell surface area accompanied by up-regulation of ANF- mRNA. Similarly, treatment of cardiomyocytes with harmine, a specific inhibitor of DYRK1A, revealed cardiomyocyte hypertrophy on morphological and molecular level. Moreover, constitutively active calcineurin led to robust induction of an NFAT-dependent luciferase reporter, whereas DYRK1A attenuated calcineurin-induced reporter activation in cardiomyocytes. Conversely, both knockdown and pharmacological inhibition of DYRK1A significantly augmented the effect of calcineurin in this assay. In summary, we identified DYRK1A as a novel negative regulator of cardiomyocyte hypertrophy. Mechanistically, this effect appears to be mediated via inhibition of NFAT transcription factors.