Epicardial Research Articles

Hydropericardium in hypothyroidism. Features of the clinic, diagnosis and treatment

Thyroid hormone deficiency negatively affects the state of the cardiovascular system, causing the formation of a «myxedematous heart». The frequency and clinical features of primary hypothyroidism in 84 patients discharged from the endocrinology department of the RCH in 2017-2022 were retrospectively analyzed. Pericardial effusion was registered in 9 people (10.7%). Massive effusion with separation of pericardial leaflets from 30 to 50 mm and the threat of tamponade was in 4 out of 9 patients. Even with a large volume of pericardial effusion, classic symptoms such as shortness of breath, orthopnea, chest pain, and a feeling of fullness were absent or poorly expressed. Differential diagnosis with diseases accompanied by accumulation of fluid in the pericardial cavity was required. Indicators of thyroid status made it possible to associate the hydropericardium with decompensation or hypothyroidism for the first time. Thyroid hormone replacement therapy led to the disappearance of hypothyroidism symptoms and a decrease in the hydropericardium. With a high risk of tamponade in 3 patients, therapy was supplemented with pericardiocentesis. A clinical observation of successful treatment with thyroid hormones in combination with pericardiocentesis of severe newly diagnosed hypothyroidism against the background of autoimmune thyroiditis with massive pericardial effusion is presented.

Pacemaker lead has penetrated the myocardium into the pericardial cavity. Pacemaker lead oscillates in the pericardial cavity as the heart beats. Moderate to large PE was seen in the pericardial cavity.

Pacemaker lead has penetrated the myocardium into the pericardial cavity. Pacemaker lead oscillates in the pericardial cavity as the heart beats. Moderate to large PE was seen in the pericardial cavity.

Pacemaker lead is observable in the RVOT. Pacemaker lead oscillates in the RVOT as the heart beats. Moderate to large PE was seen in the pericardial cavity.

Pacemaker lead is observable in the RVOT. Pacemaker lead oscillates in the RVOT as the heart beats. Moderate to large PE was seen in the pericardial cavity.

Enlarging Pericardial Thrombus Thwarted in Time - A Case Report

An acute haemopericardium due to central venous catheter (CVC) insertion can be catastrophic if not recognised promptly. In the tight confines of the pericardial sac, the haemopericardium, coupled with the rare presentation of a rapidly enlarging pericardial thrombus, can propel a hitherto reversible event into an irreversible one. In the highly possible event of cardiac arrest, with the massive thrombus compressing the right ventricle, the chance of attaining return of spontaneous circulation (ROSC) is dim due to ineffective ventricular re-filling during cardiopulmonary resuscitation. In the presence of a thrombus, pericardiocentesis will be futile. Furthermore, the likelihood of re-occurrence of cardiac arrest is relatively high even if ROSC is attained. We report a case of rapidly-growing pericardial thrombus in a post-cardiac arrest patient with acute haemopericardium following CVC insertion. Timely recognition of enlarging pericardial clots causing pericardial tamponade, with the aid of point-of-care-ultrasonography, is crucial, and emergency pericardiotomy improves the failure-to-rescue index in such patients.

Study of Postmortem Pericardial Fluid Concentrations of Troponin T (cTnT) in Sudden Natural Death

Background: Cardiovascular diseases are the most common cause of sudden natural deaths. Cardiac troponin T is a specific biochemical marker of myocardial injury. It may help in diagnosis of early myocardial ischemia when macroscopic or microscopic evidence of necrosis is not available after autopsy. Aim of this study was to assess the effectiveness of pericardial fluid troponin T in predicting the cause of death in sudden natural death and in estimating the post mortem interval. Materials and methods: It was a pilot study. Sudden natural deaths brought to Forensic Medicine department of GMCH Nagpur for autopsy were included in this observational cross sectional study. Deaths were grouped into cardiac and non cardiac deaths. Pericardial fluid samples were collected by opening pericardial sac with sterile syringe and processed on Roche Cobas e411 autoanalyzer for estimation of levels of Troponin T. Results: There was a statistically significant difference seen for the values between the groups (p<0.05) for Trop-T (ng/ml) with higher values in Cardiac cause. There was a statistically non significant correlation between postmortem interval vs Trop T (p>0.05). Conclusion: Estimation of pericardial fluid Troponin-T may be used to predict the cause of death in sudden natural death.

Posterior pericardiotomy and the prevention of post-operative atrial fibrillation and cardiac tamponade in isolated coronary artery bypass grafting – A retrospective analysis

BackgroundPost-Operative Atrial Fibrillation (POAF) is the most frequent complication of cardiac surgery and is associated with reduced survival, increased rates of cognitive changes and cerebrovascular accidents, heart failure, renal dysfunction, infection, length of stay and hospital costs. Cardiac tamponade although less common, carries high morbidity and mortality. Shed mediastinal blood in the pericardial space is a major source of intrapericardial oxidative stress and inflammation that triggers POAF. The utilisation of a posterior pericardiotomy (PP) aims to shunt blood from pericardium into the pleural space and have a role in the prevention of POAF as well as cardiac tamponade.Methods2168 patients had undergone isolated Coronary Artery Bypass Grafting at Royal Hobart Hospital from 2008 to 2022. They were divided into PP group vs. control group. Patient baseline demographics, intraoperative data and post-operative outcomes were reviewed retrospectively.ResultsTotal incidence of new POAF and cardiac tamponade was 24% and 0.74% respectively. Primary outcome of both the incidence of POAF (20.2% vs. 26.3%, p < 0.05) and Cardiac Tamponade (0% vs. 1.1%, p < 0.05) were less in the pericardiotomy group. A subgroup analysis of patients with recent myocardial infarction showed reduced incidence of POAF in the PP group (p < 0.05). Increasing age, Body Mass Index, poor left ventricular ejection fraction (EF < 30%) and return to theatre were independent predictors of developing POAF. There were similar rates of return to theatre for bleeding however, no cases of tamponade in the pericardiotomy group. There were no complications attributable to left posterior pericardiotomy and the time added to the duration of surgery was minimal.ConclusionPosterior pericardiotomy is associated with a significant reduction in the incidence of POAF and cardiac tamponade which is safe and efficient.

Ventricular Epicardial Adipose Distribution on Human Hearts: 3-Dimensional Reconstructions and Quantitative Assessments.

Epicardial interventions have forged new frontiers in cardiac ablation and device therapies. Healthy human hearts typically present with significant adipose tissue layers superficial to the ventricular myocardium and may hinder success or increase the complexities of epicardial interventions. We quantitatively evaluated the distribution of epicardial adipose tissue on the surface of human hearts and provided high-fidelity 3-dimensional reconstructions of these epicardial adipose tissue layers. The regional thickness of adipose tissues was analyzed at 51 anatomical reference points surrounding both ventricles and compared to specific patient demographics. Adipose deposits on the human hearts displayed characteristic patterns, with the thickest accumulations along the interventricular septa (anterior, 9.01 ± 0.50mm; posterior, 6.78 ± 0.50mm) and the right ventricular margin (7.44 ± 0.57mm). We provide one of the most complete characterizations of human epicardial adipose location and relative layer thickness. These results are considered fundamental for an underlying anatomic understanding when performing procedures within the pericardial space.

Blood or Fat? Differentiating Hemopericardium versus Epicardial Fat Using Focused Cardiac Ultrasound.

Basic point-of-care ultrasound of the heart-also known as Focused Cardiac Ultrasound (FoCUS)-has emerged as a powerful bedside tool to narrow the differential diagnosis of causes of hypotension. The list of causes of hypotension that a FoCUS provider is expected to be able to recognize includes a compressive pericardial effusion due to hemopericardium (blood in the pericardial sac). But hemopericardium can be difficult to distinguish from a more common condition that is not immediately life-threatening: epicardial fat. This paper reviews illustrative images of both epicardial fat and hemopericardium to provide practice guidance to the FoCUS user on how to differentiate these two phenomena.

Sudden death due to spontaneous rupture of the right atrium in the setting of chronic lung disease: A case report and review.

Sudden death from haemopericardium as a result of a right atrial rupture is uncommon, most particularly when this occurs spontaneously without any prior trauma or evidence of atrial wall pathology. The deceased was a 32-year-old man. At lunchtime his symptoms of unease, giddiness and unconsciousness began and within 45 minutes he arrived at the hospital. His vitals could not be recorded in the emergency department, and after CPR, he was pronounced dead. At autopsy, an isolated right atrial rupture, without any disease of the heart wall, was discovered. The right atrium has the weakest wall and is frequently the site of spontaneous rupture brought on by increased intraluminal pressure. Both liquid blood and clotted blood were found in the pericardial cavity. Low atrial pressure encourages clot formation because it causes considerably slower blood entry into the pericardium at the time of atrial rupture compared with entry at the time of ventricular rupture. Evidence of chronic lung disease was found which explains the raised intraluminal pressure of the heart chambers. Even with no history of trauma or myocardial infarction, the Beck triad - an engorged neck vein, a muffled heart sound, and low blood pressure - should alert the emergency room staff to the possibility of cardiac tamponade because, in a very unlikely scenario, spontaneous cardial wall rupture might occur.

Use of autologous pericardial patch graft for treatment of large congenital peritoneopericardial diaphragmatic hernia in two cats

AbstractPeritoneopericardial diaphragmatic hernia is a congenital structural anomaly that results in a communication between the pericardial sac and the peritoneal cavity. About 50% of cats are asymptomatic at the time of diagnosis, and the remaining animals may show respiratory (dyspnoea, tachypnoea, exercise intolerance), gastrointestinal, cardiac tamponade or non‐specific clinical signs. Median celiotomy for herniorrhaphy without tension (primary closure, muscle flaps, synthetic mesh, pericardium) is the treatment of choice for symptomatic patients. Two cats were referred with respiratory signs, secondary to a peritoneopericardial diaphragmatic hernia. A pericardial patch graft was obtained and sutured to the diaphragm as an alternative technique to close the large defect and not cause excessive tension on the diaphragm and caval foramen. Patients recovered well without any complications after surgery. Re‐examination and thoracic radiographs at 1 week, 2 weeks, 3 months, 6 months and 3 years postoperatively showed no signs of recurrence and the patients remained asymptomatic.

Use of three-dimensional electroanatomic mapping for epicardial access: needle tracking, electrographic characteristics, and clinical application.

Pericardiocentesis is usually completed under fluoroscopy. The electroanatomic mapping (EAM) system allows visualizing puncture needle tip (NT) while displaying the electrogram recorded from NT, making it possible to obtain epicardial access (EA) independent of fluoroscopy. This study was designed to establish and validate a technique by which EA is obtained under guidance of three-dimensional (3D) EAM combined with NT electrogram. 3D shell of the heart was generated, and the NT was made trackable in the EAM system. Unipolar NT electrogram was continuously monitored. Penetration into pericardial sac was determined by an increase in NT potential amplitude and an injury current. A long guidewire of which the tip was also visible in the EAM system was advanced to confirm EA. Epicardial access was successfully obtained without complication in 13 pigs and 22 patients. In the animals, NT potential amplitude was 3.2 ± 1.0 mV when it was located in mediastinum, 5.2 ± 1.6 mV when in contact with fibrous pericardium, and 9.8 ± 2.8 mV after penetrating into pericardial sac (all P ≤ 0.001). In human subjects, it measured 1.54 ± 0.40 mV, 3.61 ± 1.08 mV, and 7.15 ± 2.88 mV, respectively (all P < 0.001). Fluoroscopy time decreased in every 4-5 cases (64 ± 15, 23 ± 17, and 0 s for animals 1-4, 5-8, 9-13, respectively, P = 0.01; 44 ± 23, 31 ± 18, 4±7 s for patients 1-7, 8-14, 15-22, respectively, P < 0.001). In five pigs and seven patients, EA was obtained without X-ray exposure. By tracking NT in the 3D EAM system and continuously monitoring the NT electrogram, it is feasible and safe to obtain EA with minimum or no fluoroscopic guidance.

Extrapleural pneumonectomy for sarcoma: Outcomes of adult patients at a specialized center.

Extrapleural pneumonectomy (EPP) is a complex surgical procedure involving en-bloc resection of the parietal and visceral pleura, lung, pericardium, and ipsilateral diaphragm. Small case series of pleural-based sarcoma of predominantly pediatric patients suggest EPP may be a life-prolonging surgical option. We aimed to describe the characteristics and outcomes of adults who underwent EPP at a specialized sarcoma center. Clinicopathologic variables, surgical details, and follow-up information were extracted for patients undergoing EPP for pleural-based sarcoma between August 2017 and December 2020. Primary outcomes were event-free survival (EFS) and overall survival (OS) from the date of EPP. Secondary outcomes were disease-free interval (DFI) prior to EPP, and early and late postoperative complications. Eight patients were identified, seven with soft tissue sarcoma and one with bone sarcoma. Patients had either localized disease with a primary thoracic sarcoma, sarcoma recurrent to the thorax, or de novo metastatic disease. All patients underwent resection of their pleural-based sarcoma by an experienced cardiothoracic surgeon, and some patients had pre or postoperative treatment. The perioperative morbidity was comparable with previously published reports of EPP performed in mesothelioma patients. At median follow-up of 22.5 months, median EFS was 6.0 months and OS was 20.7 months. Six patients (75%) had disease recurrence; five (62.5%) died of progressive disease. Two patients (25%) had not recurred: one died of a radiation-related esophageal rupture, and one was alive with no evidence of disease at 37.0 months. Characteristics of those with the longest EFS included low-grade histology and achieving a metabolic response to preoperative chemotherapy. In adults with pleural-based sarcoma, EPP is rarely curative but appears to be a feasible salvage procedure when performed at specialized centers. Patient selection is critical with strong consideration given to multimodal therapy to optimize patient outcomes. In the absence of a confirmed response to neoadjuvant treatment, long term survival is poor and EPP should not be recommended.

Epicardial Space: Comprehensive Anatomy and Spectrum of Disease.

The epicardial space (ES) is the anatomic region located between the myocardium and the pericardium. This space includes the visceral pericardium and the epicardial fat that contains the epicardial coronary arteries, cardiac veins, lymphatic channels, and nerves. The epicardial fat represents the main component of the ES. This fat deposit has been a focus of research in recent years owing to its properties and relationship with coronary gossypiboma plaque and atrial fibrillation. Although this region is sometimes forgotten, a broad spectrum of lesions can be found in the ES and can be divided into neoplastic and nonneoplastic categories. Epicardial neoplastic lesions include lipoma, paraganglioma, metastases, angiosarcoma, and lymphoma. Epicardial nonneoplastic lesions encompass inflammatory infiltrative disorders, such as immunoglobulin G4-related disease and Erdheim-Chester disease, along with hydatidosis, abscesses, coronary abnormalities, pseudoaneurysms, hematoma, lipomatosis, and gossypiboma. Initial imaging of epicardial lesions may be performed with echocardiography, but CT and cardiac MRI are the best imaging modalities to help characterize epicardial lesions. Due to the nonspecific onset of signs and symptoms, the clinical history of a patient can play a crucial role in the diagnosis. A history of malignancy, multisystem diseases, prior trauma, myocardial infarction, or cardiac surgery can help narrow the differential diagnosis. The diagnostic approach to epicardial lesions should be made on the basis of the specific location, characteristic imaging features, and clinical background. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

The evaluation of simultaneous combined surgery of the heart and thyroid - own experience

Background: Patients referred for cardiac surgery usually also have other comorbidities. These include thyroid disease, which is relatively common in patients with heart problems. Objective: The aim of the study was to evaluate the feasibility of combined cardiac surgery and thyroidectomy. Material and methods: Six patients (age range: 54-74 years) qualified for elective, simultaneous surgeries on the heart and the thyroid were included in the study. In preparation for thyroidectomy, three patients were diagnosed with diseases requiring cardiac surgery. Two patients, initially referred for coronary artery bypass surgery in one case, and surgery for recurrent fluid in the pericardial sac with concomitant mitral and tricuspid valve regurgitation and atrial fibrillation in the other, were diagnosed in large hyperactive goitre. The last patient with a history of oncology (lung tumour), admitted to the Department of Cardiology due to a heart attack, had a CT scan of the chest, which revealed retrosternal goitre narrowing the lumen of the trachea to 9 mm. All patients underwent combined heart and thyroid operation and were discharged home 8-27 days after surgery. Conclusions: Although preoperative qualification requires a very cautious and individual approach, simultaneous surgeries in patients requiring thyroid and heart interventions are expedient and should be promoted.

An ecotoxicological assessment of a strigolactone mimic used as the active ingredient in a plant biostimulant formulation

A risk assessment on the aquatic toxicity of the plant biostimulant strigolactone mimic (2-(4-methyl-5-oxo-2,5-dihydro-furan-2-yloxy)-benzo[de]isoquinoline-1,3-dione (SL-6) was performed using a suite of standardised bioassays representing different trophic groups and acute and chronic endpoints. In freshwater, three trophic groups of algae, crustacea and fish were used. Whilst in seawater, algae (unicellular and macroalgae), Crustacea and Mollusca were employed. In addition, the genotoxicity of SL-6 was determined with the comet assessment performed on unicellular marine algae, oysters, and fish embryos. This was the first time ecotoxicity tests have been performed on SL-6. In freshwater, the lowest LOEC was measured in the unicellular algae at 0.31 mg/L SL-6. Although, similar LOEC values were found for embryo malformations and impacts on hatching rate in zebrafish (LOEC 0.31–0.33 mg/L). Consistent malformations of pericardial and yolk sac oedemas were identified in the zebrafish embryos at 0.31 mg/L. In marine species, the lowest LOEC was found for both Tisbe battagliai mortality and microalgae growth at an SL-6 concentration of 1.0 mg/L. Significant genotoxicity was observed above control levels at 0.0031 mg/L SL-6 in the unicellular algae and 0.001 mg/L SL-6 in the oyster and zebrafish larvae. When applying the simple risk assessment, based on the lowest NOECs and appropriate assessment factors, the calculated predicted no effect concentration (PNEC), for the ecotoxicity and the genotoxicity tests were 1.0 µg/L and 0.01 µg/L respectively.

Abstract 3832: Fluid-overload associated large B-cell lymphoma and determinants of outcomes: Analysis of a pooled database

Abstract Background: Fluid overload-associated large B-cell lymphoma (FOLBCL) is a new WHO lymphoma entity previously known as PEL-like lymphoma or HHV8-unrelated PEL-like lymphoma. It is a rare lymphoma that arises within fluid-filled body cavities without a solid component. FOLBCL occurs in the absence of immunodeficiency and is commonly associated with conditions associated with fluid overload. It expresses mature B-cell phenotype and occasional EBV infection markers. We conducted this study to delineate key clinicopathological characteristics, prognostic indicators, and treatment modalities that affect outcomes in this rare and newly defined lymphoma subtype. Materials &amp; Methods: To study the demographic characteristics, molecular and immunohistochemical signatures, therapeutic interventions, survival, and prognostic factors, we compiled a pooled database of 115 cases that fit the diagnostic criteria for FOLBCL. Kaplan-Meier survival curves were constructed. Cox proportional hazards model and Log-rank tests were used to assess the influence of demographic and clinicopathologic factors on overall survival (OS). Results: A total of 115 patients with confirmed FOLBCL were identified. The median age was 74, with a peak incidence between 72 and 82 years. There was a male preponderance with M:F of 2. The pleural, pericardial, and peritoneal cavities were involved in 75%, 34, and 27%. The median duration of symptoms before diagnosis was 1 month. Patients presented with constitutional symptoms and hepatosplenomegaly in 13% and 1%, respectively—no patients presented with lymph node and bone marrow involvement. The median OS of the whole group was 16 months. Patients younger than 70 had better median OS than 70 and older (24 vs. 10 months, p=0.008). The presence of any malignant peritoneal effusion was associated with worse OS when compared to effusion limited to the thoracic cavity (7 vs. 24 months, p=0.005). LDH&amp;gt;500 was also associated with worse OS (p=0.02). Sex, the presence of fluid overload-causing conditions, and serum IL2R levels did not seem to impact OS. MYC re-arrangement had numerically worse OS when present but did not reach statistical significance. Chemotherapy demonstrated superior OS compared to no treatment (24 vs. 7 months, p=0.009). Patients who attained CR as their best response had a superior median OS (p&amp;lt;0.0001). Conclusion: This study presents updated clinicopathologic data from a pooled cohort of patients with FOLBCL. It identifies the age, the primary location of cavitary disease, LDH, active therapy, and quality of response to treatment as key determinants of OS. Citation Format: Philip A. Haddad, Supriya Gupta, Christopher Graham. Fluid-overload associated large B-cell lymphoma and determinants of outcomes: Analysis of a pooled database [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 1 (Regular Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(6_Suppl):Abstract nr 3832.

Cardiac Tamponade Caused By Carbon Dioxide Insufflation During Robot-Assisted Thoracic Surgery: A Case Report.

Various complications can occur during robot-assisted thoracic surgery for mediastinal tumors owing to carbon dioxide (CO2) insufflation. This study reports the case of a 43-year-old woman who underwent robot-assisted surgery for an anterior mediastinal tumor with the subxiphoid approach. Shortly after starting CO2 insufflation, the blood pressure decreased significantly. Subsequent examination of the mediastinum revealed a left-sided pericardial injury. Cardiac tamponade due to entry of CO2 gas into the pericardial cavity was suspected. A deliberate incision was made in the right pericardium, ultimately resolving the cardiac tamponade and substantially improving the patient's blood pressure.

Giant Coronary Artery Aneurysm Associated with Extraluminal Migration of Stent: Role of Multimodal Imaging

Abstract Extraluminal migration of a drug-eluting intracoronary stent into pericardial space with or without aneurysm formation is a rare complication. It is usually symptomatic although rare asymptomatic cases have been described. Herein, we report a 49-year-old male who presented with atypical chest pain 9 years after intracoronary stent implantation in the right coronary artery and the ramus intermediate branch. Echocardiographic examination showed a 7.4 cm × 3.8 cm large oblong cystic mass along the lateral wall of the left ventricle with inward bulging of the adjacent segments. Computed tomography and invasive coronary angiography showed this mass to be a partly thrombosed giant oblong coronary aneurysm arising from the bifurcation of the left main coronary artery with extraluminal migration of the stent from the ramus branch.

Pericarditis. Causes, Mechanisms of Development, Variants of Course and Therapeutic Approaches in Modern Conditions

Under physiological conditions, the pericardial cavity contains serous fluid (15-50 ml), which belongs to the plasma ultrafiltrate and is visualized during echocardiography only in systole. The thickness of the pericardium in an adult averages 2 mm (1-3 mm). Inflammatory lesions of the pericardium with or without effusion into its cavity can act as an independent disease, and as a secondary manifestation of another pathological condition. Pericarditis of infectious (14-16%) and non-infectious (15-20%) nature, primary and secondary, acute, chronic and recurrent are commonly distinguished. The prevalence of acute and idiopathic recurrent pericarditis is 27.7 per 100,000 population and 5.4-8.1 per 100,000 population, respectively. Currently, idiopathic recurrent pericarditis belongs to polygenic autoinflammatory diseases, where an important role in the initiation of the pathologic process belongs to the inflammatory cytokine — interleukin-1 (β and α). The most characteristic manifestations of acute pericarditis are chest pain, dyspnea and fever. The article summarizes the main aspects of etiology, clinical manifestations, diagnosis and therapy of pericarditis, including the peculiarities of its treatment taking into account specific conditions. A clinical case demonstrated a case of acute effusion pericarditis with recurrence, which was characterized by rapid progression of heart failure symptoms, as well as systemic manifestations (anemia, hypercytokinemia, marked muscle weakness). The results of echocardiography allowed to identify the presence of pericardial effusion. Steroid and non-steroidal anti-inflammatory, anticoagulant and antibacterial therapy led to the disappearance of pericardial effusion and normalization of the activity of inflammatory markers in serum. However, in the dynamics of observation the attempt to suspend glucocorticoid therapy again led to fluid accumulation in the pericardium, which required continuation of steroidal and non-steroidal anti-inflammatory drugs.

Nile Tilapia (Oreochromis niloticus) Patched1 Mutations Disrupt Cardiovascular Development and Vascular Integrity through Smoothened Signaling.

Hedgehog (Hh) signaling is crucial in cardiovascular development and maintenance. However, the biological role of Patched1 (Ptch1), an inhibitory receptor of the Hh signaling pathway, remains elusive. In this study, a Ptch1 ortholog was characterized in Nile tilapia (Oreochromis niloticus), and its function was investigated through CRISPR/Cas9 gene knockout. When one-cell embryos were injected with CRISPR/Cas9 targeting ptch1, the mutation efficiency exceeded 70%. During 0-3 days post fertilization (dpf), no significant differences were observed between the ptch1 mutant group and the control group; at 4 dpf (0 day after hatching), about 10% of the larvae showed an angiogenesis defect and absence of blood flow; from 5 dpf, most larvae exhibited an elongated heart, large pericardial cavity, and blood leakage and coagulation, ultimately dying during the 6-8 dpf period due to the lack of blood circulation. Consistently, multiple differentially expressed genes related to angiogenesis, blood coagulation, and heart development were enriched in the ptch1 mutants. Furthermore, Smoothened (Smo) antagonist (cyclopamine) treatment of the ptch1 mutants greatly rescued the cardiovascular disorders. Collectively, our study suggests that Ptch1 is required for cardiovascular development and vascular integrity via Smo signaling, and excessive Hh signaling is detrimental to cardiovascular development.