Coronary artery compression by dilated pulmonary artery in an early infantile case of tetralogy of Fallot with absent pulmonary valve: ventricular fibrillation as the initial symptom of myocardial ischaemia.

Coronary artery compression by a dilated pulmonary artery is a rare complication in patients with tetralogy of Fallot with absent pulmonary valve. We present a case in which this condition manifested at two months of age, with ventricular fibrillation as the initial symptom of myocardial ischaemia. It is important to recognise that this potentially fatal complication can occur in early infancy.

Myocardial ischemia in the setting of extrinsic coronary compression: two case reports.

Background: Myocardial ischemia occurs in the setting of inadequate or complete cessation of blood supply to the myocardium. While atherosclerosis is the most common cause; other causes have been identified. Rare cases can be caused by extrinsic compression of the coronaries by a dilated pulmonary artery (PA) or by mechanical obstruction from nearby chest tubes or drains. We present two cases of myocardial ischemia-driven malignant arrhythmia leading to cardiac arrest caused by obstruction of the coronary blood flow from external compression. Case Description: In the first case, venous bypass graft compression from a chest tube postoperatively was noted and in the second case left main coronary artery (LMCA) compression from a dilated PA secondary to pulmonary artery hypertension (PAH) was seen. Diagnosis of these two cases was made via emergent coronary angiogram and intravascular ultrasound (IVUS) and treated by placing a drug eluting stent (DES) in LMCA compression and by adjusting the chest tube and placing a DES in the venous bypass graft with the restoration of flow. We also review the available literature regarding the incidence, diagnosis, and management of this rare entity. Conclusions: Overall, extrinsic compression of the coronaries is rare, therefore clinicians need to be aware of this infrequent process, to allow for appropriate diagnosis, management, and to prevent excess morbidity and mortality from this rare complication.

Histopathology of resected tissue from repair of anomalous aortic origin of a coronary artery: Potential mechanism of coronary artery compression.

Objective: This study aimed to describe the histomorphologic characteristics of resected (unroofed) common wall tissue from repair of anomalous aortic origin of a coronary artery and to determine whether the histologic features correlate with clinical and imaging findings. Methods: The histology of resected tissue was analyzed and reviewed for the presence of fibrointimal hyperplasia, smooth muscle disarray, mucoid extracellular matrix accumulation, mural fibrosis, and elastic fiber disorganization and fragmentation using hematoxylin and eosin and special stains. Clinical, computed tomography imaging, and surgical data were correlated with the histopathologic findings. Results: Twenty specimens from 20 patients (age range, 7-18 years; 14 males) were analyzed. Anomalous aortic origin of a coronary artery involved the right coronary in 16 (80%), and a slit-like ostium was noted in 18 (90%). By computed tomography imaging, the median proximal coronary artery eccentricity index was 0.4 (range, 0.20-0.90). The median length of intramural course was 8.2 mm (range, 2.6-15.2 mm). The anomalous vessel was determined to be interarterial in 14 patients (93%, 15 had evaluable images). The median distance from a commissure was 2.5 mm above the sinotubular junction (STJ) (range: 2 mm below the STJ-14 mm above the STJ). Prominent histopathologic findings included elastic fiber alterations, mural fibrosis, and smooth muscle disarray. The shared wall of the aorta and intramural coronary artery is more similar to the aorta histologically. Mural fibrosis and elastic fiber abnormalities tended to be more severe in patients >10 years of age at the time of surgery, but this did not reach statistical significance. The extent of vascular changes did not appear to have a clear relationship with the imaging features. Conclusions: The findings confirm the aortic wall-like quality of the intramural segment of the coronary artery and the presence of pathologic alterations in the wall microstructure.

Unusual Case of Acute Coronary Syndrome Due to Compression of the Left Main Coronary Artery From a Contained Aortic Root Perforation.

We present an intriguing and rare case of a 71-year-old male who presented with a non-ST elevation myocardial infarction (NSTEMI). Initial coronary angiography revealed severe and unusual systolic extrinsic compression of the left main coronary artery (LM), warranting further advanced imaging investigations. Computed tomography angiography (CTA) and transesophageal echocardiography (TEE) were employed to determine the underlying cause, which was identified as a contained aortic rupture leading to the formation of a pseudoaneurysm in the left coronary sinus of Valsalva and aortic root. This condition was found to be a sequela of previously undiagnosed endocarditis, likely secondary to lower extremity osteomyelitis and bacteremia, for which the patient received prolonged intravenous (IV) antibiotic therapy. This case highlights the critical role advanced imaging techniques play in accurately diagnosing and characterizing complex cardiovascular abnormalities, enabling early intervention and optimizing patient outcomes. Healthcare providers should remain vigilant for such atypical presentations to ensure timely and appropriate management.

Treatment of left main coronary artery compression in the setting of unpalliated congenital heart disease: a case series.

Background: Left main coronary artery compression syndrome (LMCS) is a well-characterized phenomenon resulting from compression of the left main coronary artery (LMCA) between the aorta and an enlarged pulmonary arterial trunk. The development of LMCS is usually described in the context of severe pulmonary arterial hypertension. Cases of LMCS, in the context of unpalliated congenital heart disease (CHD), are complex clinical scenarios that challenge traditional treatment paradigms. Case summary: Here, we discuss two thought-provoking patients with unpalliated CHD complicated by severe pulmonary hypertension (PH). Both patients developed LMCS, one with severe non-ST elevation myocardial infarction and the other with refractory angina. Their pulmonary vascular resistance was severely elevated despite pulmonary vasodilator therapy, and concomitant surgical correction of their CHD in addition to bypass grafting was deemed high risk. They underwent successful percutaneous coronary intervention (PCI) of the LMCA with drug-eluting stents. Discussion: Pulmonary hypertension can develop in the setting of long-standing unpalliated CHD. Surgical correction of congenital heart defects may be performed in select patients with systemic-to-pulmonary shunts, contingent on the status of PH severity. Pulmonary vasodilator therapy modulates haemodynamics to ensure surgical correction without risk of cardiopulmonary demise-termed the 'treat and repair' strategy. LMCS, an increasingly recognized phenomenon in patients with long-standing PH, is a notable complicating factor in the 'treat and repair' strategy. We introduce the concept that PCI of the LMCA may bridge patients to corrective surgery for CHD by allowing time for optimization of their pulmonary vasodilator therapy.

Phasic Diastolic Coronary Artery Compression: A Rare Cause of Chest Pain at Rest.

Phasic diastolic coronary artery compression (PDCAC) is a rare phenomenon caused by the compression of a coronary artery between expanding myocardium and a non-compliant overlying structure. We report a unique case of an elderly female who presented with recurrent paradoxical substernal chest pain at rest caused by PDCAC of the proximal left circumflex artery (LCx). Her chest pain likely occurred at rest due to longer diastolic compression time at slower heart rates. Pericardial adhesion secondary to past breast radiation was the likely cause of PDCAC. She was treated successfully with oral anti-hypertensive and anti-anginal medical therapy. PDCAC is a rare phenomenon but should be on the differential for chest pain occurring at rest, especially if there is a history of mediastinal or cardiac radiation or inflammation. PDCAC treatment depends on the underlying cause but can be treated successfully with medical therapy alone.

Dehiscent Prosthetic Aortic Valve and Aortic Root Pseudoaneurysm Complicated by Left Main Coronary Artery Compression.

Prosthetic aortic valve dehiscence is an uncommon complication of prosthetic valve endocarditis that may occur in patients who have undergone aortic valve replacement (AVR). The concurrent presence of aortic root pseudoaneurysm may further complicate the clinical presentation through the external compression of coronary arteries. Thus, patients may present with clinical features of coronary ischemia. Echocardiogram and coronary angiography are useful in establishing diagnosis. Treatment involves a multidisciplinary approach involving cardiologists, infectious disease specialists, and cardiothoracic surgeons. The authors of this study discuss a 51-year-old male who presented with anginal chest pain and was found to have a new left bundle branch block, elevated troponins, and left main coronary artery compression complicating aortic root aneurysm. He ended up requiring a re-do AVR, repair of the pseudoaneurysm, and coronary artery bypass graft.

Case report: Acute ST-elevation myocardial infarction and cardiogenic shock caused by a giant right sinus of Valsalva aneurysm and right coronary artery compression.

A sinus of Valsalva aneurysm (SVA) is a rare aortic disease that may be congenital or acquired. Patients with an intact SVA are usually asymptomatic, whereas a ruptured SVA may cause acute chest pain and dyspnea. We present a rare case of acute ST-elevation myocardial infarction and cardiogenic shock in a 51-year-old man. Emergency coronary angiography revealed a giant aneurysm with an absence of flow in the right coronary artery. Both two-dimensional echocardiography and computed tomography angiography showed a giant right SVA, which ruptured into the pericardial sac and led to extrinsic compression of the right coronary artery. Surgical repair combined with coronary bypass grafting was performed. Unfortunately, the patient died from low cardiac output syndrome and postoperative multiple organ failure. This case highlights that the possibility of SVA rupture should be considered in acute myocardial infarction cases and that echocardiography and coronary computed tomography angiography are important in providing an accurate and rapid SVA diagnosis.

The Clinical Trial Outcomes of Med-Zenith PT-Valve in the Treatment of Patients With Severe Pulmonary Regurgitation.

Objective: Nearly 2/3 of patients with dilated right ventricular outflow tract (RVOT) were excluded from pulmonary valves transplantation due to the lack of size-matched valves. Here, we explored the safety and efficacy of the Med-Zenith PT-Valve for the treatment of patients with severe pulmonary regurgitation. Methods: 22 Patients with severe PR (grade 3+,4+) were enrolled based on the anatomical features of native RVOT and the valve design. The immediate, 3-months and 1-year post-procedural follow-up data were analyzed. Results: The baseline mean systolic diameters in the distal main pulmonary artery (MPA), MPA sinus junction, MPA sinus, pulmonary annulus, RVOT aneurysm and muscular outlet measured with computed tomography were 33.6 ± 6.1, 34.0 ± 5.8, 37.9 ± 6.0, 32.4 ± 7.3, 41.9 ± 9.3, and 34.4 ± 8.0 mm, respectively. The PT-Valve landing zone was set within these levels. Successful valve implantations were achieved in all patients without noticeable device malposition, coronary artery compression, pulmonary branch obstruction or paravalvular leak during follow-ups. Post-procedural pulmonary artery diastolic pressure increased from 5.8 ± 3.1 to 11.3 ± 2.5 mmHg. In the 3-month and 1-year follow-up, the right ventricular end diastolic volume index reduced from the baseline 181.6 ± 29.0 to 143.7 ± 29.7 ml/m2 and 123.4 ± 31.2 ml/m2, and the trans-pulmonary valve gradient decreased from 25.6 ± 22.2 to 10.64 ± 3.54 mmHg and 11.16 ± 3.0 mmHg, respectively. The 6-min walk distance increased from 416.6 ± 97.9 to 455.9 ± 64.6 m and 467.8 ± 61.2 m, respectively. Conclusion: This clinical trial revealed favorable outcomes for the safety, efficacy and feasibility of the Med-Zenith PT-Valve in the treatment of severe PR with significantly enlarged RVOT.

Left main coronary artery compression by dilated pulmonary artery in pulmonary arterial hypertension: a systematic review and meta-analysis.

OBJECTIVE: Pulmonary arterial hypertension (PAH) can lead to left main coronary artery compression (LMCo), but data on the impact, screening and treatment are limited. A meta-analysis of LMCo cases could fill the knowledge gaps in this topic. METHODS: Electronic databases were searched for all LMCo/PAH studies, abstracts and case reports including pulmonary artery (PA) size. Restricted maximum likelihood meta-analysis was used to evaluate LMCo-associated factors. Specificity, sensitivity and accuracy of PA size thresholds for diagnosis of LMCo were calculated. Treatment options and outcomes were summarized. RESULTS: A total of five case-control cohorts and 64 case reports/series (196 LMCo and 438 controls) were included. LMCo cases had higher PA diameter (Hedge's g 1.46 [1.09; 1.82]), PA/aorta ratio (Hedge's g 1.1 [0.64; 1.55]) and probability of CHD (log odds-ratio 1.22 [0.54; 1.9]) compared to non-LMCo, but not PA pressure or vascular resistance. A 40 mm cut-off for the PA diameter had balanced sensitivity (80.5%), specificity (79%) and accuracy (79.7%) for LMCo diagnosis, while a value of 44 mm had higher accuracy (81.7%), higher specificity (91.5%) but lower sensitivity (71.9%). Pooled mortality after non-conservative treatment (n = 150, predominantly stenting) was 2.7% at up to 22 months of mean follow-up, with 83% survivors having no angina at follow-up. CONCLUSION: PA diameter, PA/aorta ratio and CHD are associated with LMCo, while hemodynamic parameters are not. Data from this study support that a PA diameter cut-off between 40 and 44 mm can offer optimal accuracy for LMCo screening. Preferred treatment was coronary stenting, associated with low mid-term mortality and symptom relief. Diagnosis and management of left main coronary artery compression (LMCo) in patients with pulmonary arterial hypertension (PAH).

Eisenmenger syndrome with left main compression syndrome: a case report.

BACKGROUND: Left main coronary artery disease secondary to pulmonary artery compression related to Eisenmenger syndrome is an under-suspected condition that can cause fatal outcomes if left untreated. It presents with typical angina but is frequently mistaken for pulmonary hypertension (PH) symptoms. It is now recognized as one of the few important causes of angina in PH. CASE PRESENTATION: A 37-year-old man with a history of unoperated atrial septal defect and Eisenmenger syndrome came to the outpatient department with a chief complaint of angina on exertion. Electrocardiogram showed regular sinus rhythm with right axis deviation, right ventricular hypertrophy, deep T-wave inversion in inferior and anterior leads suggestive of ischemia or strain, and incomplete right bundle branch block. Cardiac CT showed compression of the left main coronary artery due to a dilated main pulmonary artery. Therefore, this patient was diagnosed with Eisenmenger syndrome with left main compression due to dilated pulmonary artery. He was treated successfully with IVUS-guided stent implantation. The patient experienced marked improvement in regular activities, with no recurrence of angina symptoms. Angiography 3 months after the procedure revealed good patency of the stent, without significant stenosis. CONCLUSIONS: Left main coronary artery compression is a complication that should be suspected in patients with Eisenmenger syndrome presenting with angina symptoms. Non-invasive modalities are recommended for diagnostic evaluation, but the gold-standard technique remains coronary angiography. The best treatment is not well-established, with either myocardial revascularization or PH treatment, but a left main coronary artery stenting procedure is considered an ideal emergent treatment to provide a better quality of life for patients in this condition.

2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Developed in collaboration with and endorsed by the Heart Rhythm Society (HRS), the American College of Cardiology (ACC), the American Heart Association (AHA), and the Association for European Paediatric and Congenital Cardiology (AEPC) Endorsed by the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS).

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.