Pulmonary valve stenosis is a congenital heart disease that is usually diagnosed during childhood, while presentation in adulthood is uncommon and may lead to delayed diagnosis. We report the case of a 21-year-old patient with no significant medical history who presented with a six-month history of exertional dyspnea and constrictive chest pain. Electrocardiography showed right axis deviation with signs of right ventricular hypertrophy, and chest radiography revealed dilatation of the left pulmonary artery. Transthoracic echocardiography demonstrated severe valvular pulmonary stenosis with a peak velocity of 4.53 m/s and a maximum systolic gradient of 82.23 mmHg, associated with right ventricular hypertrophy and preserved systolic function, without evidence of pulmonary hypertension. Computed tomography pulmonary angiography confirmed focal post-stenotic dilatation of the left pulmonary artery. After a multidisciplinary discussion, balloon pulmonary valvuloplasty was recommended. This case highlights the importance of considering congenital pulmonary valve stenosis in young adults presenting with exertional symptoms and underscores the challenges of access to advanced interventional therapies in resource-limited settings.

Double dorsal aorta is a rare congenital embryological vascular anomaly resulting from incomplete regression and fusion of the dorsal aortae during embryogenesis. This anomaly could be associated with various other congenital heart defects (CHDs), including transposition of the great arteries, ventricular septal defect (VSD), persistent truncus arteriosus, tetralogy of Fallot (TOF), and Coarctation of the aorta (COA). This is a 9 year old girl with complex congenital heart disease and a double dorsal aorta who underwent a single ventricle palliation and this anomaly was detected during pre Fontan evaluation. She presented at age of 2 weeks with cyanosis, initial diagnosis was situs inversus, Dextrocarida, DORV with malposed great arteries, subpulmonary and valvular pulmonary stenosis, Patent ductal arteriosus and left superior vena cava. Patient was discussed with cardiothoracic team and Underwent left bidirectional Glenn at 10 months of age (2017) During pre-Fontan catheterization (June 2021) → incidental discovery of double descending (double dorsal) aorta, subsequently confirmed by CTA Fontan Completion: Deferred due to multiple severe comorbidities. Double dorsal aorta is an extremely rare embryologic vascular anomaly due to failure of fusion of the paired dorsal aortae. Although was asymptomatic, its identification is important in complex congenital heart disease because it may affect surgical planning and catheter based interventions. In this patient, the anomaly was detected incidentally during pre Fontan evaluation emphasizing the value of detailed preoperative imaging.

Management of late presenters with cyanotic congenital heart disease (CCHD) and reduced pulmonary blood flow is challenged by multi-system derangements from longstanding hypoxia in addition to anatomic factors. Balloon pulmonary valvuloplasty (BPV) is a potentially viable palliative option for selected patients with severely stenotic pulmonary valve and are unsuitable for definitive surgical repair METHODS: A retrospective chart review was conducted (2002-2024, age >12 years). BPV was considered for those with a significant component of valvular pulmonary stenosis (PS) on echocardiography. A strategy of stepwise dilation using progressively larger balloon sizes, andmonitoring pulmonary artery pressures between each dilation, was adapted. Thirty patients [TOF (n = 13), DORV/DILV (n = 7/2), single ventricle/Unbalanced AVCD (n = 3), and corrected transposition (n = 5)] with median age: 20.5 years (IQR: 14.0-26.8), weight: 49 kg (IQR: 44.1-57.7) underwent BPV. The fluoroscopy and procedure times were 17.8 min (IQR:12.7-25.7) and 71 ± 26 min (mean ± SD), respectively. The balloon-to-annulus ratio was 1.01 (0.96-1.2). Saturation improved significantly from 76.7 ± 9.6 % to 90.2 ± 4.4 %. Complications included transient arrhythmias in five patients and fatal re-perfusion pulmonary edema during repeat BPV in one. At a median follow-up of 51 months (IQR: 26.75-139.25 months), majority of patients demonstrated sustained symptomatic relief. Seven patients underwent corrective surgery, and four required repeat BPV. In a selected subgroup of anatomically suited patients with CCHD and PS, stepwise dilatation of the pulmonary valve resulted in improved oxygenation and symptomatic relief, which was sustained on follow-up, making it an attractive low-resource option for palliation, particularly in resource constrained LMIC settings.

Papillary thyroid carcinoma (PTC) is the most common thyroid malignancy. Pulmonary valve stenosis (PVS), on the other hand, is a congenital valvular heart disease characterized by narrowing of the right ventricular outflow tract, which can lead to right-sided heart failure if severe. To date, there are no reported cases in the literature describing a co-occurrence of PTC and PVS in the same individual. Our patient was a 28-year-old male, a smoker with a five-pack-year history, who presented with a painless right neck swelling of five months duration. Physical examination revealed a firm, non-tender right cervical lymphadenopathy. Thyroid examination showed that the trachea was midline, thyroid was non-palpable, without tenderness, nodules, or bruit, and no signs of exophthalmos or proximal muscle weakness. Heart examination revealed a thrill, a normal first and second heart sounds, and a systolic crescendo-decrescendo murmur heard at the left upper sternal border. Jugular venous pressure (JVP) was not elevated. No signs of ascites, hepatomegaly, or lower limbs edema. The patient had no syndromic features. Laboratory wise was unremarkable. In regard to imaging, neck ultrasound showed multiple enlarged heterogeneous rounded structures in the right cervical region, the largest measures 2.9 x 2.5 cm. The visualized parts of the thyroid gland appeared unremarkable. Computed tomography (CT) of the brain, chest, abdomen, and pelvis was unremarkable apart from cardiomegaly in the CT chest. Further assessment by echocardiography showed severe valvular pulmonary stenosis. An ultrasound-guided fine needle aspiration of the right cervical lymph node showed papillary thyroid carcinoma. A thorough literature review using PubMed revealed no documented cases of concurrent PTC and PVS, supporting the novelty of this case. While the possibility of a coincidental coexistence cannot be excluded, this unique case raises the possibility of a shared developmental or molecular pathway-such as dysregulation of the rat sarcoma (RAS)/mitogen-activated protein kinase (MAPK) signaling cascade-linking both conditions. While a coincidental occurrence remains plausible, the overlap with pathways implicated in RASopathies warrants further investigation. Multidisciplinary evaluation was critical in planning appropriate oncologic and cardiologic management. This case underscores a rare coexistence of papillary thyroid carcinoma and severe pulmonary valve stenosis in a young adult without any identifiable congenital syndrome. Although the association may be incidental, it prompts consideration of potential shared developmental or molecular mechanisms. Importantly, the presence of severe pulmonary stenosis has significant clinical implications, contributing to symptom burden and influencing the timing and selection of therapeutic interventions, including both cardiac and oncologic management strategies.

Long-term right ventricular outflow tract dysfunction can lead to right and left heart failure. Nonetheless, current data on the clinical characteristics of the right ventricular outflow tract obstruction (RVOTO) in China remain limited. This study analyzed the evolving trends in the observed proportion, etiology, spectrum, and interventions for RVOTO over the past 18 years from a single-center echocardiographic database. A total of 10,234 RVOTO cases (17,451 records from 2003 to 2020) were included in the transthoracic echocardiography database in China. The RVOTO cases were divided into eight groups according to the different obstruction sites and disease types. Subsequently, RVOTO was categorized into three types: congenital, iatrogenic, and neither congenital nor iatrogenic. Moreover, congenital RVOTO was further classified into simple and complex congenital RVOTO. Next, we calculated the proportion of RVOTO patients who had received interventions. We analyzed the proportions of different types of RVOTO and the variation tendency. During 2003-2008, 2009-2014, and 2015-2020, the observed proportion of RVOTO cases in the total echocardiographic cases decreased (3.2%, 2.1% and 1.8%, respectively; p < 0.001); the proportion of RVOTO with a congenital etiology also reduced, though as the dominant cause; meanwhile, the proportion of simple congenital RVOTO increased (48.5%, 52.4% and 67.3%, respectively; p < 0.001). As the two most common types of RVOTO, the proportion and number of valvular pulmonary stenosis (PS) increased, while the proportion of Fallot cases decreased. The number of RVOTO cases receiving surgical or transcatheter intervention and experiencing intervention-related severe pulmonary regurgitation (PR) or residual peripheral PS increased, although with a low probability of reoperation. The clinical characteristics of RVOTO have undergone significant changes in China over the past two decades. After the RVOTO intervention, the increasing number of cases with severe PR or residual peripheral PS and a low possibility of reoperation signifies a future necessity for transcatheter pulmonary intervention.