Sinus of Valsalva aneurysm dissecting the inter­ ventri­cular septum with rupture into the right ven­ tricle (RCD code: I 1B.O)

Introduction Coronary artery fistula (CAF) is an anomaly in which abnormal connections are present between the coronary artery and the cardiac chambers or a major vessel. It is an uncommon anomaly with an estimated incidence of 1 in 50,000 live births and usually occurs in isolation. We report a case of CAF into right atrium in a patient who admitted to our outpatient department with dyspnea and fatique. With help of transthoracic echocardiography (TTE), transesophageal echocardiography (TOE) and cardiac computerized tomography (CCT) we put the definite diagnosis and decided to close this fistula percutaneously with a PDA occluder. Case A 43-year- old male admitted to our outpatient department with complaints of exertional dyspnea and fatique for 5 years. His physical examination revealed a holosystolic murmur on the aortic valve area. His TTE showed enlarged right heart chambers with a high pulmonary artery systolic pressure (50 mmHg) and a suspicious flow from the aortic root into right atrium. Qp/Qs was 2.1. TOE depicted a shunt between aorta and right atrium as well, we couldn’t truly demonstrate the connection though . In order to define the defect precisely, we performed a cardiac CT. Cardiac CT clearly showed a markedly dilated and mildly tortuous and calcified fistula arising from the osteal part of right coronary artery draining into right atrium. Right coronary artery was thin and there was no stenosis. Cardiac CT helped us to exclude coronary arter disease as well. As it was suitable to close percutaneously, we decided to close it with a PDA occluder. Conclusion CAF is a rare, generally congenital anomaly and may cause right heart chamber dilatation and pulmonary hypertension if the diagnosis is missed. It is important to support and clarify the underlying pathology with help of other cardiovascular imaging modalities like cardiac CT and cardiac magnetic resonance imaging (CMR), if TTE and TOE cannot demonstrate us the exact pathology.In our case our choice of extra method was cardiac CT, as we wanted to exclude accompanying coronary artery disease at the same time. In today’s era, the use of multimodality imaging is increasing with a tremendous rate and it helps clinical cardiologists, cardiovascular imaging specialists and interventional cardiologists all. Abstract P1490 Figure. 2D,3D TOE and CCT images of CAF

Rupture of sinus of Valsalva aneurysm (SVA) is a fatal complication, and early detection and immediate surgical correction is critical for lifesaving. However, achieving its definite diagnosis is challenging. Computed tomography (CT) can provide excellent diagnostic value for SVA rupture and other combined anomalies with better spatial resolution of cardiac structure. Herein, we described a case of a young woman with ruptured SVA. SVA rupture was suggested by transthoracic echocardiography and confirmed by cardiac CT. CT scan confirmed SVA rupture by revealing a ring-like structure originating from the right coronary sinus of the aortic valve protruding to the right ventricle with extravasation of the radiocontrast agent. This finding was not clearly defined by transthoracic echocardiography. Cardiac CT may be useful for the diagnosis of ruptured SVA by providing more detailed visualization of adjacent structures and additional information on combined cardiac pathology.

Left main coronary artery (LMCA) atresia is a rare congenital heart disease and can be fatal in pediatric patients. We report an adult case of LMCA atresia, in which heart failure developed without episodes suggesting angina. A 40-year-old man presented with difficulty breathing. Echocardiography revealed diffuse hypokinesis of the left ventricle with an ejection fraction of 22% in the absence of significant valvular disease. A diagnosis of heart failure was made, and diuretics, enalapril, bisoprolol and warfarin were administered. Coronary angiography demonstrated no trace of the ostium of the LMCA in the sinuses of Valsalva; the middle to distal part of the LMCA was visualized by rich collateral flow from the right coronary artery to the left anterior descending coronary artery and left circumflex coronary artery. No trace of the ostium of the LMCA from the aorta or main pulmonary artery was detected on computed tomography angiography or echocardiography. The patient underwent coronary artery bypass grafting and a final diagnosis of congenital atresia of LMCA was made. The clinical course was uneventful and computed tomography angiography, performed 5 days after surgery, showed a patent bypass graft. This case demonstrates the importance of considering LMCA atresia even in the absence of chest symptoms suggesting angina in patients with heart failure. J Med Cases. 2021;12(6):233-237 doi: https://doi.org/10.14740/jmc3681

Sinus venosus atrial septal defects (SVASDs), concurrent with partial anomalous pulmonary venous connections (PAPVCs), are a rare congenital heart disease in dogs. Surgical correction is essential when clinical signs or significant hemodynamic changes are present. We aimed to report on the successful surgical correction of an SVASD with PAPVCs, using a computed tomography (CT)-based customized 3D cardiac model. A 10-month-old male poodle was referred for corrective surgery for an ASD. Echocardiography confirmed a hemodynamically significant left-to-right shunting flow through an interatrial septal defect and severe right-sided heart volume overload. For a comprehensive diagnosis, a CT scan was performed, which confirmed an SVASD with PAPVCs. A customized 3D cardiac model was used for preoperative decision-making and surgical rehearsal. The defect was repaired using an autologous pericardial patch under a cardiopulmonary bypass (CPB). Temporary pacing was applied for sinus bradycardia and third-degree atrioventricular block. The patient recovered from the anesthesia without further complications. The pacemaker was removed during hospitalization and the patient was discharged without complications 2 weeks post-surgery. At the three-month follow-up, there was no shunting flow in the interatrial septum and the right-sided volume overload had been resolved. The cardiac medications were discontinued, and there were no complications. This report indicates the validity of surgical correction under CPB for an SVASD with PAPVCs, and the advantages of utilizing a CT-based 3D cardiac model for preoperative planning to increase the surgical success rate.

Left ventricular outflow tract obstructions (LVOTOs) encompass a series of stenotic lesions starting in the anatomic left ventricular outflow tract (LVOT) and stretching to the descending portion of the aortic arch (Figure 1). Obstruction may be subvalvar, valvar, or supravalvar. These obstructions to forward flow may present alone or in concert, as in the frequent association of a bicuspid aortic valve with coarctation of the aorta. All of these lesions impose increased afterload on the left ventricle and, if severe and untreated, result in hypertrophy and eventual dilatation and failure of the left ventricle. LVOTOs are congenital in the vast majority of individuals younger than 50 years in the United States; some variants of subaortic obstruction are the exception. It is imperative to consider all patients with LVOTO at a high risk for developing infective endocarditis, and one should always institute appropriate measures for prophylaxis. The present article is intended as a contemporary review of the causes, manifestations, treatments, and outcomes of LVOTO; it will not address LVOTO in the pediatric population or genetic hypertrophic cardiomyopathy but will focus strictly on congenital malformations in the adult. Figure 1. Artist’s rendering of the LVOTO lesions in sequence as viewed from a superolateral orientation. A, Gradient echo cardiac MR image as viewed from the frontal projection demonstrating flow acceleration at a site of supravalvar aortic stenosis (white arrow) in a patient with Williams syndrome. The black arrow identifies the level of the unrestricted aortic valve. B, Classic radiological signs of coarctation of the aorta: rib notching (white arrows) as seen on a posteroanterior chest x-ray in a patient with coarctation of the aorta. The rib notching is caused by erosion of the inferior rib margins by dilated pulsatile posterior intercostals collateral arteries. The black arrow points to the Figure 3 silhouette that …

PH Grand Rounds: A Case of Pulmonary Hypertension Associated With High Cardiac Output State from Arteriovenous Fistula–Or Is It?

Factors associated with the risk of heart failure progression in patients with non-compaction cardiomyopathy

Sinus of Valsalva aneurysms (SVAs) are rare. We assessed the role of multimodality imaging in guiding the contemporary management. A single-center retrospective cohort study over a 20-year period was performed. Between January 1997 and June 2017, 103 patients were diagnosed with SVAs (median age: 58 years). Eighty patients presented with non-ruptured SVAs, and 23 with ruptured SVAs. Seventy-six patients underwent surgery, and 27 were conservatively managed. The median durations of follow-up were: 48 months (surgical group) vs. 37.5 months (conservative group). There was no mortality directly attributable to SVA surgery. There were no late complications in the conservative group. Transthoracic echocardiography (TTE) was the first-line imaging investigation (100.0% in surgical group vs. 92.6% in conservative group, P=0.019). Additional imaging studies included: (I) transesophageal echocardiography (TEE): 93.4% in surgical group vs. 22.2% in conservative group, P<0.001; (II) multi-detector cardiac computed tomography (MDCT): 61.8% in surgical group vs. 37.0% in conservative group, P=0.041; (III) cardiac magnetic resonance (CMR): 22.4% in surgical group vs. 14.8% in conservative group, P=0.579. At diagnosis, SVA diameters were: TTE: 4.80 cm (range, 3.30 cm); TEE: 5.40 cm (range, 4.00 cm); MDCT: 5.20 cm (range, 3.90 cm); CMR: 4.80 cm (range, 3.70 cm). In a 20-year cohort, proper selection for surgery and conservative management resulted in excellent outcomes for SVAs. TTE was the first-line imaging investigation for assessment of SVAs, although many patients underwent an additional imaging investigation. The contemporary outcomes of imaging-guided SVA management were excellent.

A Case of Ruptured Giant Sinus of Valsalva Aneurysm Into the Right Ventricle in a Patient With Both Atrial and Ventricular Septal Defects

Quadricuspid aortic valve (QAV) is a rare congenital heart disease, ranking behind bicuspid and unicuspid aortic valves in terms of the incidence of congenital aortic valve abnormalities. When symptoms are present, they are typically related to aortic regurgitation, manifesting as shortness of breath, nocturnal dyspnea, and palpitations, or aortic stenosis, which presents with exertional dyspnea, angina, or syncope. We present the case of an asymptomatic male, diagnosed with a QAV during a routine examination. Despite the absence of clinical symptoms, a thorough general physical examination, including cardiac auscultation, revealed signs suggestive of valvular abnormality. The diagnosis was confirmed via transthoracic echocardiography. For QAV, the diagnostic process typically includes transthoracic echocardiography, which allows for initial confirmation of the valve morphology. In addition, computed tomography (CT) coronary angiography provides detailed information on valvular morphology and helps identify any associated coronary stenosis. In cases of QAV, a CT aortogram is also crucial to assess potential aortic root dilation, a known complication of this congenital anomaly. This case emphasizes the importance of routine physical examination in diagnosing rare congenital heart conditions like QAV, even in asymptomatic individuals. Early diagnosis and appropriate imaging are essential for timely management, particularly in preventing the progression of associated complications such as aortic regurgitation or root dilation.

Introduction/Background: Sinus of Valsalva aneurysms are very rare congenital heart disease. Most cases of ruptured sinus of Valsalva aneurysms (RSVA) are associated with other congenital defects. We present a case of Sinus of valsalva aneurysm (SoVA) rupture with underlying supracristal VSD (sVSD). Case Presentation: A 27-year-old male with a past medical history significant for a VSD (diagnosed at childhood but lost to follow up at 18 years of age) who was admitted to the intensive care unit for management of undifferentiated shock, acute renal failure and acute liver failure. Upon initial evaluation, vital signs showed blood pressure of 130/34 mmHg, heart rate 118 bpm, SpO2 100% on 3L NC. An emergent transthoracic echocardiogram with color doppler showed unrestricted left to right flow from the RSoV to RVOT in systole and diastole with concern for ruptured SoV aneurysm. A non-contrast CT scan of the chest also showed a dilated pulmonary trunk with a connection between the SoV and RVOT. Patient underwent primary closure of the VSD, as well as a patch repair of a large fistula between R sinus of Valsalva and RVOT. Patient steadily improved with normalization of renal function and hepatic function. Discussion: Aneurysm of the sinus of Valsalva (SoV) is a rare acquired or congenital anomaly predominantly affecting the right and the noncoronary sinuses, with an estimated prevalence of 0.09% of the population. Most sinus of Valsalva aneurysms arise from the right or the noncoronary sinuses. The most common site of rupture of SOV aneurysm is into the right ventricle followed by right atrium, left ventricle, and the left atrium. Hemodynamically, the flow through VSD produces Venturi effect “windsock effect. This left to right shunt caused by the supracristal VSD weakens the elastic lamina of the aortic sinus creating the SOV aneurysm. Overtime due to continuous negative pressure a SOVA develops on the right aortic root, which can rupture into the right ventricular outflow tract as seen in our case. Conclusion: Sinus of Valsalva aneurysm rupture is a rare but potentially fatal complication and should be considered on the differential in a young individual with underlying sVSD presenting with hemodynamic collapse.

Occlusion of Left Coronary Ostium With a Rudimentary Aortic Cusp

Funding Acknowledgements Type of funding sources: Private hospital(s). Main funding source(s): Helios Clinical Research Institute Background/Introduction Patients with severe tricuspid regurgitation (TR) are characterized from severe dilatation and negative remodeling of the right heart chambers causing functional right ventricular (RV) failure and increased mortality and morbidity. The transcatheter tricuspid edge-to-edge repair (TTVR) is a new treatment option associated with symptom improvement and reduced hospitalization. The cardiac magnetic resonance imaging (CMRI) remains the gold standard for evaluating the right ventricular morphology and function and could be a useful in assessing the RV reverse remodeling after TTVR. The Tricuspid Regurgitation REgistry (TRuE) is an ongoing national prospective register of patients with severe TR that aims to identify morphofunctional parameters of the right heart chambers related to improve outcomes after TTVR. Purpose The present is a subanalysis of patients with serial CMRI examinations recruited in the ongoing prospective TRuE registry. Methods After excluding patients with intracardiac RV leads, seven patients (age 70 ±12 years) with completed thirty-day follow up were included in the present analysis. Retrospective gated steady state free precession cine images were acquired in the long and short-axis views covering the entirety of both ventricles. Aortic and pulmonary flow data were acquired with a flow-sensitive gradient echo sequence. Assessment of left ventricular (LV) and RV volumes was performed manually in the short-axis cine images (Circle Cardiovascular Imaging, Calgary, Alberta, Canada). The TR fraction was calculated as: ({total RV stroke volume (SV) – total pulmonary forward flow}/total RVSV) × 100. All volumes and flow measurements were indexed for body surface area and expressed in ml/m2. The ratio between the diameter of pulmonary artery (PA) and ascending aorta (AA) was used as indirect measurement of pulmonary arterial hypertension (PAH). CMRI parameters assessing TR quantification, RV morphology and function were analyzed at baseline and follow-up with Fisher´s test or paired t-test. Results At thirty days there was a significant reduction of the TR fraction (45% to 20%, p &amp;lt; 0.001), RV dilatation (RV-end diastolic volume (EDV): 106 ± 12ml/m2 to 86 ± 9.4ml/m2, p = 0.002, RV- end sistolic volume (ESV):55 ± 6.5 to 43 ± 6.6ml, p = 0.003) and improvement of function (RVEF: 47 ± 3.6 to 53 ± 2.4 %, p = 0.006). On the left heart side, there was an improvement of LV filling, with increased LVEDV and LVESV and of the LVSV (36 ± 5.3 to 41 ± 3.7ml/m2, p = 0.040), causing an improvement of the cardiac index (2.2 ± 0.9 l/min/m2 to 2.9 ± 0.7 l/min/m2; p = 0.019. Furthermore, the PA/AA ratio improved significantly (0.91 ± 0.15 to 0.81 ± 0.14, p = 0.006). Conclusion TTVR is associated with positive reverse remodeling of the RV with reduction of dilatation, PAH and function improvement.

Double Inlet-Double Outlet Right Ventricle (DI-DORV) is a rare and unique single-ventricular congenital heart disease with variable atrioventricular valve morphology and myocardial structure. In patients of DI-DORV the two atria are connected to the RV by two atrioventricular (AV) valves or a common set of AV valve. The right ventricle is identified by its anatomical markers (thick trabeculae, interventricular-marginal trabeculae, and Lance's muscle), and the left ventricle by the smooth endocardium surface of the basal interventricular septum. Due to different forms of the right ventricular myocardium, hypertrophy of muscle trabeculae may be mistaken for ventricular septum, which may be challenging for diagnosing DI-DORV. The patients of DI-DORV often die early due to volume overload or persistent cyanosis leading to gradual deterioration of ventricular function. Patients are often prone to recurrent chest infections since childhood, seriously affecting physical development and causing congestive heart failure at an early stage. However, the complexity and diversity of DI-DORV lesions lead to great difficulty in clinical diagnosis. The most common surgical treatment is a staged modified Fontan palliative surgery. Transthoracic echocardiography (TTE) can accurately and reliably estimate the cardiac structure and function and plays an essential role in diagnosing and managing patients with DI-DORV. Furthermore, utilising the current non-invasive imaging techniques like cardiac CT and cardiac MRI, in situations where echocardiography is unable to deliver a precise diagnosis, are essential for optimal surgical management to improve the survival rates and quality of life. Here we are presenting a case report of a deeply cyanotic 3-month-old male infant afflicted with complex congenital cardiac defect: double inlet-double outlet right ventricle (DI-DORV) associated with A-malposition of great arteries and severe pulmonary valvular stenosis.

Pathophysiology of Systolic and Diastolic Heart Failure