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.

Diagnosis difficulties in elderly patients with atrial fibrillation and complete atrioventricular block

The association of atrial fibrillation (AF) with complete atrioventricular block (CAVB) is a common clinical feature in elderly patients. It is characterized by the loss of specific symptoms of AF (palpitations, intermissions); in the first place may come CAVB symptoms: dizziness, Morgagni–Adams–Stokes (MAS) attacks. Aim. The article objective is to illustrate the dynamic changes in the course of AF with the development of CAVB on the example of a clinical case and to discuss the difficulties in timely diagnosis and therapy correction. Case presentation. A 75-year-old male was diagnosed with a rapid ventricular response form of AF. The onset of CAVB caused the transition from rapid ventricular response form of AF to slow ventricular response form, which was initially accompanied by a subjective improvement in the patient’s condition. As the disease progressed, the patient’s condition worsened due to the development of MAS attacks. The elderly patients with a permanent form of AF require constant cardiac monitoring by an experienced specialist who has appropriate vigilance in management of patients with arrhythmias, awareness about possible concomitant conduction disorders. This provides comprehensive cardiac support, including timely pacemaker implantation which gives more options for AF symptoms monitoring and heart rate control, has a positive modifying effect on drug therapy. Conclusions. Changes in the clinical picture of AF after development of CAVB can lead to late diagnosis of CAVB, inadequate therapy and untimely pacemaker implantation, as a consequence, to progression of concomitant pathology and the development of life-threatening complications, as in our clinical case.

A surgical approach of an unusual variant of complete atrioventricular defect; A case report

Background Complete atrioventricular canal is a congenital heart defect that is characterized by an atrial septal defect, ventricular septal defect, and a common atrioventricular valve. Standard surgical techniques for repairing complete atrioventricular canal defect mainly includes repairing the defect with a single patch, a double patch, or with the modified single patch technique. Case presentation This paper presents a novel surgical repair technique of an unusual anatomical presentation for a complete atrioventricular canal defect in a patient with Down syndrome. Conclusions Unusual anatomical variant for congenital heart defects occurs frequently, which gives surgeons real opportunities to innovate surgical approaches. This patient was an example of an unusual anatomical presentation for complete atrioventricular canal, and the surgical technique used for this patient was novel. Follow up for these patients is mandatory for long term results.

A case of complete atrioventricular block with extremely high blood concentration of azelnidipine

Background Azelnidipine, a dihydropyridine calcium channel blocker (CCB), has less adverse effects (e.g. hot flushes and reflex tachycardia) compared to other dihydropyridine CCBs. Azelnidipine has been reported to reduce heart rate as opposed to inducing tachycardia. No evidence of bradycardia or complete atrioventricular block (CAVB) with azelnidipine treatment has been reported. Case presentation In the present study, a 92-year-old woman was diagnosed with CAVB while taking azelnidipine and simvastatin for an extended period of time, and referred to our medical center. It was thought that the CAVB may have been an adverse effect of azelnidipine treatment. Specifically, it was considered that in this patient, one of the causes might be the concomitant use of simvastatin inhibiting the metabolism of azelnidipine by cytochrome P450 enzyme 3A4. Consequently, it was suggested to the patient’s physician that the patient’s serum azelnidipine levels be measured and treatment with azelnidipine and simvastatin be discontinued. The patient’s serum concentration of azelnidipine at the time of her visit to our center was 63.4 ng/mL, higher than the normal acceptable level. There was no occurrence of CAVB for 4 weeks, to present, following discontinuation of azelnidipine and simvastatin treatment. Conclusions Azelnidipine has a different mechanism of action that other CCBs. In very rare cases, it may cause CAVB when combined with CYP3A4 inhibitors. If a patient taking azelnidipine is diagnosed with CAVB, physicians should suspect that the condition may be an adverse effect of azelnidipine and should consider discontinuing azelnidipine. And, in the elderly, it is necessary to avoid concomitant use of CYP3A4 inhibitors.

Successful Prevention of Fetal Autoimmune-Mediated Heart Block by Combined Therapies With Hydroxychloroquine and Intravenous Immunoglobulin: A Case Report

A fetal autoimmune-mediated atrioventricular block is a passively acquired autoimmune disease in which maternal autoantibodies enter the fetal circulation via the placenta and subsequently cause inflammation and fibrosis of the atrioventricular node. Once fetal autoimmune-mediated atrioventricular block occurs, it only takes a short time to progress from first-degree atrioventricular block to complete atrioventricular block, meaning that the damage is often irreversible. Autoimmune—associated AVB, a rare but life—threatening disorder, occurs in 2–5% of pregnancies with positive anti—Ro/SSA (the most common one) and La/SSB antibodies. The perinatal mortality of neonates with AVB outlined in research is approximately 30%. Thus far, for autoimmune-associated AVB fetuses, currently used treatments include corticosteroids, hydroxychloroquine, intravenous immunoglobulin (IVIG), b—sympathomimetic agent, and even plasma exchange. Currently, approaches for preventing the progression and recurrence of a fetal atrioventricular block are still controversial. Here, we reported a baby of successful prevention from the fate of the fetal atrioventricular block by adopting prophylactic comprehensive prenatal therapy.

Transient Complete Heart Block after Percutaneous Transcatheter ASD Device Closure

Transcatheter device closure of atrial septal defect (ASD) is associated with increased complications in small children in device size ≥ 19mm or indexed size ≥ 0.18 mm/cm and weight≤ 15 kg. Complete atrioventricular heart block (CHB) is reported as a rare complication of ASD device closure. We report the case of a 7year-old boy, 23kg weight, underwent device closure of ASD and subsequently developed CHB next day after the procedure which was completely resolved by day 3 with steroid treatment. Similar case reports were rarely reported in the literature. Keywords: atrial septal defect, complete heart block, device/height ratio.