Patent ductus arteriosus (PDA) is a congenital defect characterized by abnormal blood flow between the aorta and pulmonary artery. Existing closure devices, such as the Amplatzer Duct Occluder (ADO), face challenges with affordability, biocompatibility, and anatomical adaptability. This study evaluates the safety and feasibility of a novel nitinol-based PDA occluder, the first developed sample in Iran, designed to enhance biocompatibility, reduce thrombogenicity, and improve durability. This preclinical study was conducted in 2024 at the Large Animal Research Laboratory, Shiraz University of Medical Sciences, Shiraz, Iran. The occluder was fabricated from custom-made nitinol wires braided into a 72-wire conical mesh, ensuring flexibility and durability. Deployment was tested in a sheep model via femoral and pulmonary artery access. Post-procedure evaluations included angiography, clinical monitoring, and histopathological analyses to assess tissue integration, thrombogenicity, and biocompatibility. The device was successfully deployed in two target sites with stable positioning and no procedural complications. Angiographic imaging confirmed vessel patency, even in an artery with a size mismatch. The animal exhibited no adverse outcomes, maintaining normal pulses and activity over a three-month follow-up. Post-mortem analysis revealed secure device placement without migration, perforation, or aneurysm. Histopathological findings demonstrated mild inflammation, neointimal formation, and re-endothelialization, with no significant thrombus or granuloma, indicating excellent biocompatibility. This study provides preliminary evidence supporting the feasibility, safety, and biocompatibility of the Iranian-developed PDA occluder. These findings suggest the device may serve as a viable, cost-effective alternative for PDA closure, addressing device shortages and advancing regional medical technology.

Granulicatella adiacens is an uncommon cause of infective endocarditis (IE) and has rarely been associated with mycotic pulmonary artery aneurysm (MPAA). An 18-year-old woman with patent ductus arteriosus and poor dental hygiene presented with massive hemoptysis. Multimodal imaging identified G. adiacens IE complicated by a saccular MPAA and severe valvular involvement. Management included targeted antibiotics, endovascular embolization, and combined mitral-aortic valve surgery with patent ductus arteriosus closure, resulting in full clinical recovery. A fastidious microbiological behavior can delay diagnosis, making multimodal imaging essential for detecting complications such as MPAA. Coordinated medical-surgical therapy is critical in complex IE presentations. G. adiacens can cause aggressive IE with rare vascular complications. Multimodal imaging is indispensable for timely detection of MPAA in IE, enabling staged interventions that optimize outcomes. Cantú syndrome is characterized by congenital hypertrichosis; distinctive coarse facial features (including broad nasal bridge, wide mouth with thick vermilion of the upper and lower lips, and macroglossia); enlarged heart with enhanced systolic function, as well as pericardial effusion and, in many, a large patent ductus arteriosus (PDA) requiring repair; and musculoskeletal abnormalities (thickening of the calvaria, broad ribs, scoliosis, flaring of the metaphyses, and low muscle tone). Other cardiovascular abnormalities may include dilated aortic root and ascending aorta with rare aortic aneurysm, tortuous vascularity involving brain and retinal vasculature, and pulmonary arteriovenous communications. Generalized edema (which may be present at birth) spontaneously resolves; peripheral edema of the lower extremities (and sometimes arms and hands) may develop at adolescence. Developmental delays are common, but intellect is typically normal; behavioral problems can include attention-deficit/hyperactivity disorder, autism spectrum disorder, obsessive-compulsive disorder, anxiety, and depression. The diagnosis of Cantú syndrome can be established in a proband with characteristic clinical and imaging findings and/or a heterozygous gain-of-function pathogenic variant in ABCC9 or KCNJ8 identified by molecular genetic testing. Treatment of manifestations: Referral to dermatologist for hypertrichosis treatment options; surgical or device closure of PDA in infancy or early childhood as needed; pericardiocentesis and pericardial stripping as needed to treat pericardial effusion; developmental and educational support; compression stockings and other standard management including lymphedema pump for peripheral edema; standard treatment for gastroesophageal reflux; individualized management for migraine headaches; management of pulmonary hypertension per cardiologist; treatment of seizures per experienced neurologist; bracing and/or surgery as needed for scoliosis. Surveillance: Annual echocardiogram and electrocardiogram to monitor cardiac size and function, as well as for evidence of pericardial effusion. Clinical evaluation and cardiac biomarkers to monitor late development of high-output cardiac failure. Monitor for a history of persistent headaches or other neurologic symptoms, which may require brain imaging for cerebral vasculature abnormality and evaluation by a neurologist. Monitor developmental progress, educational needs, and behavioral assessment at each visit. Monitor for evidence of peripheral edema annually starting in adolescence. Assess for gastroesophageal reflux disease as needed. Assess for scoliosis with physical examination, followed by spine radiographs as needed. Agents/circumstances to avoid: Minoxidil, diazoxide, and angiotensin-converting enzyme inhibitors. Evaluation of relatives at risk: Clarify the genetic/clinical status of older and younger relatives of an affected individual in order to identify as early as possible those who should be evaluated and monitored for cardiac manifestations of Cantú syndrome, as well as peripheral edema and scoliosis. Cantú syndrome is inherited in an autosomal dominant manner. Each child of an individual with Cantú syndrome has a 50% chance of inheriting a Cantú syndrome-related pathogenic variant. If the causative ABCC9 or KCNJ8 pathogenic variant has been identified in an affected family member, prenatal and preimplantation genetic testing are possible. If the Cantú syndrome-causing pathogenic variant has not been identified in an affected family member, evaluation of a pregnancy at increased risk using ultrasound to look for fetal macrosomia and polyhydramnios and a fetal echocardiogram to evaluate for cardiovascular abnormalities is recommended.

A patent ductus arteriosus (PDA) can result in pulmonary arterial hypertension (PAH) due to a left-to-right shunt. Lung transplantation (LTx) is indicated when PAH becomes refractory to medical management. We report a case of bilateral LTx (BLTx) with simultaneous aortic replacement using a donor aorta in an adult patient with PAH complicated by PDA. A 27-year-old woman was referred for an LTx evaluation. At 1-year-old, she was diagnosed with a PDA. At the time of diagnosis, PDA closure was not indicated due to severe PAH, with a pulmonary vascular resistance of 33.8 Wood units. Despite receiving maximal medical therapy, her condition progressively deteriorated. She was placed on the transplant waitlist at age 27. Since left ventricular function was preserved (ejection fraction 60%) and no complex congenital heart disease was present, bilateral lung transplantation was chosen instead of heart-lung transplantation. Preoperative computed tomography revealed a giant pulmonary artery aneurysm (PAA). At 31 years of age, she underwent BLTx with simultaneous replacement of the proximal descending thoracic aorta using a donor aortic graft under cardiopulmonary bypass to enable complete excision of the ductal tissue. The giant PAA was also repaired during the same procedure. Postoperatively, she required venoarterial extracorporeal membrane oxygenation and was successfully weaned off by postoperative day 4. After an extended rehabilitation period, the patient was discharged 153 days postoperatively and remained in good health for 16 months following transplantation. To our knowledge, this is the first reported case of LTx with aortic replacement using a donor aortic graft for the management of PDA. We believe this combined procedure may represent a feasible surgical strategy for adult patients with PAH complicated by PDA and warrants further investigation in future cases.

Infective endocarditis on a patent ductus arteriosus (PDA) is a rare but serious condition that can lead to life-threatening complications, such as mycotic aneurysms. We report a case of endocarditis on a PDA, complicated by multiple mycotic aneurysms, including a partially ruptured aneurysm of the pulmonary artery (PA) trunk. A 20-year-old female presented with progressive dyspnoea, worsening over 48 h, accompanied by fever. Echocardiography revealed a large pericardial effusion with cardiac tamponade, necessitating the drainage of 500 mL of haemorrhagic fluid. Imaging studies revealed a false aneurysm of the PA trunk, with contrast extravasation into the pericardial space. Blood cultures and pericardial fluid cultures were positive for Staphylococcus aureus. Emergency surgery involved resection of the aneurysm, ligation of the PDA, and pericardial drainage. The patient recovered after 4 weeks of intravenous antibiotics and was discharged with good clinical and biological outcomes. This case illustrates the importance of early diagnosis and management of infective endocarditis associated with congenital heart defects, as the delay can result in severe complications such as aneurysm rupture.

To characterize clinical, hemodynamic, imaging, and pathologic findings in children with pulmonary arterial hypertension (PAH) and variants in SRY-box transcription factor 17 (SOX17), a novel risk gene linked to heritable and congenital heart disease-associated PAH. We assembled a multi-institutional cohort of children with PAH and SOX17 variants enrolled in the Pediatric Pulmonary Hypertension Network (PPHNet) and other registries. Subjects were identified through exome and PAH gene panel sequencing. Data were collected from registries and retrospective chart review. We identified 13 children (8 female, 5 male) aged 1.6-16 years at diagnosis with SOX17 variants and PAH. Seven patients had atrial septal defects and 2 had patent ductus arteriosus. At diagnostic cardiac catheterization, patients had severely elevated mean pulmonary artery (PA) pressure (mean 78, range 47-124 mmHg) and markedly elevated indexed pulmonary vascular resistance (mean 25.9, range 4.9-55 WU∗m2). No patients responded to acute vasodilator testing. Catheter and computed tomography angiography imaging demonstrated atypical PA anatomy including severely dilated main pulmonary arteries, lack of tapering in third and fourth order pulmonary arteries, tortuous 'corkscrewing' pulmonary arteries, and abnormal capillary 'blush.' Several children had PA stenoses and 2 had systemic arterial abnormalities. Histologic examination of explanted lungs from 3 patients disclosed plexiform arteriopathy and extensive aneurysmal dilation of alveolar septal capillaries. SOX17-associated PAH is a distinctive genetic syndrome characterized by early onset severe PAH, extensive pulmonary vascular abnormalities, and high prevalence of congenital heart disease with intracardiac and interarterial shunts, suggesting a role for SOX17 in pulmonary vascular development.