Unilateral absence of the pulmonary artery is a rare congenital defect requiring advanced imaging for diagnosis. We present two cases with a connection between a patent ductus arteriosus remnant and the left pulmonary artery. Therapeutic approaches, including imaging findings and management strategies, are discussed to highlight diagnostic and treatment considerations.

Background: Tetralogy of Fallot with absent pulmonary valve syndrome (TOF-APVS) is a rare variant of tetralogy of Fallot marked by aneurysmal pulmonary artery dilatation and variable tracheobronchial compression. The identification of clinical indicators that predict perioperative risk in this patient population remains challenging. We aimed to review the surgical outcomes and reintervention requirements of patients with TOF-APVS over a 15-year single-center experience, with particular focus on two indicators of disease severity: preoperative mechanical ventilation (MV) and computed tomography (CT)-detected airway compression. Methods: We retrospectively reviewed 27 patients with TOF-APVS who underwent surgical repair at a single center between January 2010 and January 2025. Airway compression was assessed by CT in 20 patients. The primary outcome was the need for any reintervention during follow-up. Secondary outcomes comprised early and overall mortality and postoperative duration of intubation, ICU stay, and hospital stay. Subgroup comparisons were performed using the Fisher exact test and the Mann–Whitney U test. Results: The median age at surgery was 8 months (IQR, 4.5–16). Preoperative MV was required in 5 patients (18.5%), and CT revealed airway compression in 13 of 20 patients (65.0%). Early postoperative mortality was 7.4%, and overall mortality during follow-up was 18.5%. At least one reintervention was required in 9 patients (33.3%). Preoperative MV was significantly associated with early mortality (40.0% vs. 0%; p = 0.028) and with prolonged ICU stay (median 15 vs. 4 days; p = 0.008). CT-detected airway compression was not significantly associated with either mortality or reintervention. Conclusions: In this 15-year single-center experience with 27 patients, preoperative mechanical ventilation was associated with markedly higher early postoperative mortality and prolonged ICU stay, and may thus serve as a practical clinical indicator of disease severity in TOF-APVS. CT-detected airway compression was a frequent and often severe finding, but its association with early mortality and reintervention could not be statistically confirmed, likely reflecting the selective use of CT and small subgroup size. These findings support the integration of preoperative ventilation status into perioperative risk assessment and highlight the need for larger, preferably multicenter, studies with standardized airway assessment to clarify the prognostic role of anatomical airway compression.

Background/Objectives: Left atrial appendage isomerism (LAAI) is associated with substantial variation in venous connections and intracardiac anatomy. A subgroup of LAAI patients has L-loop ventricular topology together with a particular great artery relationship—a combination that corresponds historically to Van Praagh’s {A,L,I} segmental notation—but this subgroup has not been separately characterized. We aimed to describe the anatomical spectrum, rhythm and conduction abnormalities, biventricular repair eligibility, and surgical pathways in this subgroup. Methods: This single-center retrospective study included 15 patients with LAAI, L-loop ventricular topology, and a specific great artery relationship (aorta left-sided, pulmonary artery right-sided)—a combination corresponding historically to Van Praagh’s {A,L,I} notation—evaluated between January 2010 and December 2025. Diagnosis was confirmed independently by echocardiography and cardiac computed tomography in all patients. Anatomical suitability for biventricular repair was determined by multidisciplinary consensus. Results: Median age at presentation was 3.0 months, and median follow-up was 31.0 months. An interrupted inferior vena cava was present in all patients. The great artery relationship was aorta left-posterior/pulmonary artery right-anterior in 12 patients, aorta left-anterior/pulmonary artery right-posterior in 2, and side-by-side in 1. Ventriculoarterial connection was concordant in 8 patients, double-outlet right ventricle in 6, and double-outlet left ventricle in 1. A common atrioventricular valve was present in 7 patients. Right ventricular outflow tract obstruction was present in 7 patients. Arrhythmia was documented in 6 patients, and 2 required permanent pacemaker implantation. Ten patients (66.7%) were considered anatomically eligible for biventricular repair; repair had been completed in 4, 2 had received interim palliation, and 4 remained awaiting surgery. The remaining 4 patients followed a single-ventricle pathway. One death was confirmed, and 2 patients were lost to follow-up. Conclusions: Within LAAI, the coexistence of L-loop ventricular topology and a specific great artery relationship was associated with ventriculoarterial concordance in most patients, which may support biventricular repair. Anatomical eligibility for repair, however, did not always translate into completed repair. Larger, multicenter studies are needed to confirm these findings.

A patient with Kabuki syndrome and a parachute mitral valve was referred for mitral stenosis. Echocardiography indicated right ventricular dilatation and a suspected vertical ascending vein. Cardiac CT confirmed dual drainage of the left upper pulmonary vein and hemiazygos vein continuity. Despite echocardiographic limitations, surgery was performed. This rare case is reported due to its unique anatomical variation.