Total Anomalous Pulmonary Venous Connection in Adults: Should We Offer Surgery?

Background: We aim to determine the surgical outcomes of adult patients with total anomalous pulmonary venous connection (TAPVC) and examine the regression of pulmonary artery (PA) pressures after the procedure. Methods: We reviewed the hospital records from 2003 to 2022 and identified 49 adult patients with TAPVC. We assessed their surgical outcomes and the trend of PA pressures after the procedure. Continuous data are presented as mean ± SD or median (interquartile range) and categorical variables are presented as percentages. Results: The median age of the patients was 23 years (range 18-42) and 31 (63.3%) were male. Thirty-six patients (73.5%) had supracardiac TAPVC. The mean systolic PA pressure was 65.8 ± 16.4 mm Hg and it decreased by 47.9% (34%, 61.8%) after surgery. Moderate or more tricuspid regurgitation was seen in 27 (55.1%) patients before surgery; however, it was present in only 3 (6.1%) patients during early follow-up. There was no intraoperative or 30-day mortality, and the median hospital length of stay was six days. Long-term follow-up data were available for 29 patients with the average duration of follow-up being 5.6 years (range 6 months to 15 years) and the mean systolic PA pressures of this cohort was 29.8±7.9 mm Hg. Forty-six (93.1%) patients were asymptomatic; four women had uneventful pregnancies and delivered healthy children. Conclusion: Surgical repair of the naturally selected group of adult TAPVC patients can be performed safely with good results. Regression in flow-related pulmonary hypertension and an improvement in functional quality of life are seen in nearly all patients.

Primary or Delayed Repair for Complete Atrioventricular Septal Defect, Tetralogy of Fallot, and Ventricular Septal Defect: Relationship to Country Economic Status.

OBJECTIVE: Primary repair in the first six months of life is routine for tetralogy of Fallot, complete atrioventricular septal defect, and ventricular septal defect in high-income countries. The objective of this analysis was to understand the utilization and outcomes of palliative and reparative procedures in high versus middle-income countries. METHODS: The World Database of Pediatric and Congenital Heart Surgery identified patients who underwent surgery for: tetralogy of Fallot, complete atrioventricular septal defect, and ventricular septal defect. Patients were categorized as undergoing primary repair, repair after prior palliation, or palliation only. Country economic status was categorized as lower middle, upper middle, and high, defined by the World Bank. Multiple logistic regression models were utilized to identify independent predictors of hospital mortality. RESULTS: Economic categories included high (n = 571, 5.3%), upper middle (n = 5,342, 50%), and lower middle (n = 4,793, 49.7%). The proportion of patients and median age with primary repair were: tetralogy of Fallot, 88.6%, 17.7 months; complete atrioventricular septal defect, 83.4%, 7.7 months; and ventricular septal defect, 97.1%, ten months. Age at repair was younger in high income countries (P < .0001). Overall mortality after repair was lowest in high income countries. Risk factors for hospital mortality included prematurity, genetic syndromes, and urgent or emergent operations (all P < .05). CONCLUSIONS: Primary repair was selected in >90% of patients, but definitive repair was delayed in lower and upper middle income countries compared with high-income countries. Repair after prior palliation versus primary repair was not a risk factor for hospital mortality. Initial palliation continues to have a small but important role in the management of these three specific congenital heart defects.

The meandering intrapulmonary total anomalous pulmonary venous channel (MITAPVC)-old wine in new bottle or a new variant?

Background: Total anomalous pulmonary venous connection (TAPVC), with an intrapulmonary segment (IPV), a meandering abnormally located confluence and obligatory drainage of one lung into the other before entering the systemic circulation, is a rare anomaly and we term it as the meandering intrapulmonary TAPVC (MITAPVC). Material and methods: We report five patients with an unusual variation of the TAPVC channel. A review of literature was done to identify this association of TAPVC with an intrapulmonary vein and absence of a confluence in its usual location. Results: In our study, 4 patients with neo-confluence creation had excellent outcome while one with partial correction required catheter-based intervention, but succumbed to persistent pulmonary hypertension refractory to therapy. A literature search showed 25 additional such patients. Two groups were noted, one with isolated lesions (N = 16) and the other with heterotaxy or complex intracardiac lesions (N = 14). Of the 20 surgical interventions, only 12 survived, most of them in the isolated group (N = 10). Mortality was due to incomplete surgery (4/4), inappropriate surgery (3/3), and complete and appropriate surgery (1/11) respectively. Conclusion: The MITAPVC is often associated with heterotaxy and complex lesions. However, the isolated version is being increasingly recognised. Non-recognition or inappropriate surgical correction of MITAPVC is associated with fatal outcomes. Evaluation by a computerised tomography (CT) scan, meticulous dissection and demonstration of the entire channel, creation of a neo-confluence and appropriate palliation for the heterotaxy is the key to ensure good outcome. This is not a new entity, but deserves a separate subclassification under TAPVC. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-021-01290-2.

Critical thrombotic obstruction of transverse aortic arch in a neonate.

A 3-day-old neonate presented with features suggestive of coarctation of aorta. Echocardiography showed a large organized thrombus in the transverse arch causing obstruction to theaortic arch and carotids with partial recanalization. Patient underwent surgical thromboendarterectomy with arch reconstruction. The evaluation did not reveal any hematological abnormalities and suspected to be due to fetal thromboembolism. Patient improved well, and no neurological deficits were observed during follow-up.

Multiple cardiovascular involvement in a case of relapsing polychondritis.

Relapsing polychondritis is a multisystem disease characterized by recurrent inflammation of the cartilaginous tissue. Cardiovascular manifestations of relapsing polychondritis are rare but are the second most common cause of death in these patients. We report a case of relapsing polychondritis who underwent aortic valve replacement uneventfully but presented six months later with myocardial infarction due to bilateral coronary ostial stenosis.