Long-Term Outcome Up To 40 Years after Single Patch Repair of Complete Atrioventricular Septal Defect in Infancy or Childhood

Objectives Patients with repaired complete atrioventricular septal defect (CAVSD) represent an increasing portion of grown-ups with congenital heart disease. For repair of CAVSD, the single-patch technique has been employed first. This technique requires division of the bridging leaflets, thus, among other issues, long-term function of the atrioventricular valves is of particular concern. Methods Between 1978 and 2001, 100 consecutive patients with isolated CAVSD underwent single-patch repair in our institution. Hospital mortality was 11%. Primary endpoints were clinical status, atrioventricular valve function, and freedom from reoperation in long term. Follow-up was obtained contacting the patient and/or caregiver, and the referring cardiologist. Results Eighty-three patients were eligible for long-term follow-up (21.0 ± 8.7, mean ± standard deviation [21.5; 2.1–40.0, median; min–max] years after surgical repair). Actual long-term mortality was 3.4%. Quality of life (QoL; self- or caregiver-reported in patients with Down syndrome) was excellent or good in 81%, mild congestive heart failure was present in 16%, moderate in 3.6% as estimated by New York Heart Association classification. Echocardiography revealed normal systolic left ventricular function in all cases. Regurgitation of the right atrioventricular valve was mild in 48%, mild–moderate in 3.6%, and moderate in 1.2%. The left atrioventricular valve was mildly stenotic in 15% and mild to moderately stenotic in 2%; regurgitation was mild in 54%, mild to moderate in 13%, and moderate in 15% of patients. Freedom from left atrioventricular-valve-related reoperation was 95.3, 92.7, and 89.3% after 5, 10, and 30 years, respectively. Permanent pacemaker therapy, as an immediate result of CAVSD repair (n = 7) or as a result of late-onset sick sinus syndrome (n = 5), required up to six reoperations in single patients. Freedom from pacemaker-related reoperation was 91.4, 84.4, and 51.5% after 5, 10, and 30 years, respectively. Conclusion Up to 40 years after single-patch repair of CAVSD, clinical status and functional results are promising, particularly, in terms of atrioventricular valve function. Permanent pacemaker therapy results in a life-long need for surgical reinterventions.

Neonatal Rupture of the Tricuspid Valve and Maternal Lupus: Is There a Correlation?

Valve dysfunction is not widely recognized as a feature in newborns born to mothers with positive anti-Ro/SSA antibodies, and only scarce reports have suggested an association between rupture of the atrioventricular valve tensor apparatus and these maternal antibodies. We report the follow-up from fetal life to the time of postnatal surgery of a patient with severe tricuspid regurgitation due to a flail of the anterior tricuspid valve leaflet who was born to an anti-Ro/SSA antibodies positive mother.

Late outcome, therapy and systemic ventricular function in patients with a systemic right ventricle: data of the German National Register for Congenital Heart Defects

Background: Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure. Methods: A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included. Results: Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation. Conclusion: A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.

The impact of intraoperative pericardial three-dimensional echocardiography for the atrioventricular valve repair in the patient with congenital heart disease

Background Atrioventricular valve (AVV) regurgitation enormously affected the survival outcome of the patients with congenital heart disease (CHD). However, the image quality by use of transthoracic echocardiography has not reached a level that is sufficient, and also, three-dimensional echocardiography, which is useful to clarify complex AVV anatomy, cannot be applied for the patients less than 15kg, to guide for the AVV repair in pediatric patients. We try to show surgeons more precise three-dimensional images about an AVV by using intraoperative pericardial three-dimensional echocardiography (IP3DE) and improve the surgical outcome. Purpose To determine the efficacy of IP3DE by assessing the surgical outcome of an AVV repair and re-intervention rate. Method Eighty-five patient with CHD who underwent atrioventricular repair with significant regurgitation (Grade 2–4+) before operation were divided into two groups imaged IP3DE or not, in our hospital from 1993 to 2020. We assessed the surgical outcome and re-intervention rate between two arms and re-evaluate AVV images before surgery compared to the IP3DE. Result IP3DE was performed in forty-six patients (IP3DE group) and thirty-nine patients were not (control group). Median age at AVV repair was 3.0/2.8 years, respectively. The AVV was tricuspid (n=25), mitral (n=41), or common (n=19). The IP3DE group had a significantly higher improvement in regurgitation of AVV (IP3DE: Grade 3.2±0.3 → 1.7±0.3 vs Control: Grade 2.8±0.3 → 1.8±0.3, p<0.05). Fifty-nine percent of the IP3DE group was successful outcome (Grade<1+ after repair). There was no significant difference in the rate of re-intervention after surgery between two groups. In multivariate analysis, using IP3DE contributed to successful outcome for AVV repair (OR: 4.66, 95% CI: 1.46–14.8, p<0.01). The different and/or additional anatomical AVV findings were obtained in sixty-one percent of patients by the IP3DE. Conclusion IP3DE contributes to successful outcome for AVV repair by obtaining further information on complicated AVV anatomy in congenital heart disease. IP3DE also enables both cardiovascular surgeons and cardiologists to share the accurate and detail “surgeon's view” in the operating room for planning of AVV repair. FUNDunding Acknowledgement Type of funding sources: None.