Resumen: Consenso internacional para la nomenclatura y clasificación de la válvula aórtica bicúspide congénita y su aortopatía, con fines clínicos, quirúrgicos, intervencionistas y de investigación / Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes

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Abstract This consensus of nomenclature and classification for congenital bicuspid aortic valve and its aortopathy is evidence-based and intended for universal use by physicians (both pediatricians and adults), echocardiographers, advanced cardiovascular imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists, and researchers spanning these areas of clinical and basic research. In addition, as long as new key and reference research is available, this international consensus may be subject to change based on evidence-based data1.

Fatal Acute Myocardial Infarction With Normal Epicardial Coronary Arteries Shortly Following COVID-19 Vaccination.

Myocarditis and acute myocardial infarction (AMI) have been reported after COVID-19 messenger ribonucleic acid vaccination. Nearly all reported patients with myocarditis or AMI after COVID-19 vaccination have survived and become asymptomatic. Described herein is a previously healthy man who developed severe heart decompensation shortly after receiving a COVID-19 vaccination and died approximately 40 hours later. An autopsy disclosed massive AMI.

[Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes]. / Resumen: Consenso internacional para la nomenclatura y clasificación de la válvula aórtica bicúspide congénita y su aortopatía, con fines clínicos, quirúrgicos, intervencionistas y de investigación.

This consensus of nomenclature and classification for congenital bicuspid aortic valve and its aortopathy is evidence-based and intended for universal use by physicians (both pediatricians and adults), echocardiographers, advanced cardiovascular imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists, and researchers spanning these areas of clinical and basic research. In addition, as long as new key and reference research is available, this international consensus may be subject to change based on evidence-based data1.

Este consenso de nomenclatura y clasificación para la válvula aórtica bicúspide congénita y su aortopatía está basado en la evidencia y destinado a ser utilizado universalmente por médicos (tanto pediatras como de adultos), médicos ecocardiografistas, especialistas en imágenes avanzadas cardiovasculares, cardiólogos intervencionistas, cirujanos cardiovasculares, patólogos, genetistas e investigadores que abarcan estas áreas de investigación clínica y básica. Siempre y cuando se disponga de nueva investigación clave y de referencia, este consenso internacional puede estar sujeto a cambios de acuerdo con datos basados en la evidencia1.

Causes of Malfunction of Bioprostheses Inserted Percutaneously in the Aortic Valve Position in Patients Whose Native Aortic Valve Was Congenitally Bicuspid and Stenotic.

Transcatheter aortic valve implantation (TAVI) has brought in recent years relief of cardiac-induced symptoms to a large number of patients with aortic stenosis. Whether it is better to use TAVI for the treatment of aortic valve stenosis superimposed on a congenitally bicuspid valve has been debated in contrast to its proved usefulness in aortic valve stenosis involving a tricuspid aortic valve. From January 2020 to March 2023, surgical aortic valve replacement of TAVI valve and native aortic valve was done in 6 patients. The clinical findings of the patients and morphologic findings from the surgical specimens submitted to the cardiac pathology department were subsequently examined. All the 6 native aortic valves had bicuspid configuration. The TAVI valve in each patient was excised from 9 to 88 months (mean 36 months) after it had been implanted because of paravalvular leak in 4, severe stenosis of the prosthetic valve in 1, and bioprosthetic cuspal degeneration in 1. Prosthetic valve endocarditis was clinically suspected in 2 patients, but the specimen culture was negative. Before surgical aortic valve replacement, 3 patients experienced stroke after TAVI. All 6 patients had low hemoglobin levels (mean 9.5 mg/100 ml) and low hematocrit levels (mean 29.5%). Reticulocyte count was available in 4 patients and was increased in all (mean 3.5%). When the stenotic native aortic valve configuration is bicuspid, the raphe tends to be calcified first and located perpendicular to the flow of the blood and may prevent the ring of the caged bioprosthesis from being transferred to the aortic wall, which is a requirement for full opening of the lumen of the bioprosthesis. Thus, thorough consideration needs to be made before performing TAVI in patients whose native aortic valve is stenotic and bicuspid.

Severe Heart Failure Resulting From Healed Myocardial Infarction Preferentially Involving the Ventricular Septum and Leading to Orthotopic Heart Transplantation.

Described herein are 4 patients who underwent orthotopic heart transplant (OHT) because of heart failure caused by acute myocardial infarcts which healed. These healed infarcts were the result of preferential severe narrowing of the left anterior descending coronary artery. In all 4 cases, the myocardial infarct caused severe scarring of the ventricular septum (VS), more than that observed in the left ventricular free wall where most myocardial infarcts secondary to coronary artery narrowing typically occur.

Giant-Cell Myocarditis, Cardiac Sarcoidosis, and Orthotopic Heart Transplantation.

Described herein are 2 patients diagnosed clinically as "giant cell myocarditis." Both had short clinical courses (∼ 2 months) before lifesaving orthotopic heart transplantation (OHT). Examination of the hearts disclosed multiple widespread yellow lesions in the ventricular walls. The short clinical courses in these 2 patients are quite different from cardiac sarcoidosis, which typically has courses lasting years. In contrast to cardiac sarcoidosis, the ventricular myocardial lesions were yellow in color not white as in cardiac sarcoidosis. In conclusion, we consider giant-cell myocarditis and cardiac sarcoidosis to be different conditions and not simply different stages of the same condition.

Mitral Valve Replacement for Mitral Stenosis Secondary to Massive Mitral Annular Calcium.

Mitral annular calcium (MAC) may produce mitral stenosis (MS) if its quantity is massive. We define massive MAC as the presence of a huge quantity of calcium underlying the posterior mitral leaflet and extending across all or nearly all of the ventricular aspect of the anterior mitral leaflet. This report was prompted to emphasize the hazards of performing mitral valve replacement in patients with MS secondary to massive MAC. The clinical data and morphology of the operatively excised mitral valves from the 11 patients who had mitral valve replacement for MS secondary to massive MAC are described. Of the 11 patients, 6 died postoperatively, 5 of whom had 4+/4+ MAC. The high mortality in these patients suggests that the decision to perform mitral valve replacement needs to be carefully considered if the quantity of MAC is massive.

Examination of Non-Infected Operatively-Excised Bioprostheses in the Aortic Valve Position to Determine the Reason for their Dysfunction.

Described herein are findings in 55 non-infected bioprostheses that had been in the aortic valve position from 2 to 276 months (mean 107). The major purpose of this study was to illustrate the variable causes prompting excision of the bioprostheses. Fifty-three (96%) patients survived ≥ 30 days following the bioprosthetic excision and 50 (91%) patients lived ≥1 year postoperatively. The techniques used to explant the bioprostheses appear to vary considerably among the operating surgeons.

Massively Hypertrophied Right-Sided Heart with Hypoplastic Left-Sided Heart in a Neonate (A Rare Type of Hypertrophic Cardiomyopathy).

Described herein is a newborn boy with likely right-sided hypertrophic cardiomyopathy (HC), who survived for 18 hours after birth. At necropsy, he had a severely thickened right ventricular free wall, ventricular septum, right atrial wall and a hypoplastic left-sided heart. There was a large fossa ovale type atrial septal defect and also a patent ductus arteriosus. During peak systole, the right ventricular outflow tract was obstructed, and its contents were pushed into the thick-walled right atrium, then rapidly into the thin-walled left atrium via a large fossa ovale atrial septal defect. The contents were then pushed into the thin-walled left ventricle and finally into the small ascending aorta and into the lungs via a large patent ductus arteriosus. We were unable to find a similar published case.

Origin of the Right Coronary Artery From the left Sinus of Valsalva in a Donor Heart With Unsuccessful Repair.

Described herein is a 62-year-old man who had orthotopic heart transplantation (OHT) because of severe heart failure secondary to idiopathic dilated cardiomyopathy. Because of continued symptoms of heart failure, a coronary angiogram was performed 3 years after the OHT and it showed anomalous origin of the right coronary artery (RCA) from the left sinus of Valsalva. As a consequence, an operation was performed to transfer the origin of the RCA to the right sinus of Valsalva. Unfortunately, the lumen of the RCA clotted off shortly after the operative procedure. Because of clinical evidence of rejection, the donor heart was replaced 4 years later providing the opportunity to study the previously transferred anomalous RCA.

Acute Dissection of Epicardial Coronary Arteries Shortly Following Full Term Delivery Resulting in Acute Myocardial Infarction and Leading to Orthotopic Heart Transplantation.

Described herein is a 28-year-old woman who developed acute dissection of the left main, left anterior descending, and left circumflex coronary arteries shortly after delivering a full-term normal infant. The consequence was acute myocardial infarction leading to a severely depressed left ventricular ejection fraction (<10%) and resulting in life-saving orthotopic heart transplantation procedure not previously reported in patients with spontaneous isolated coronary dissection.

Circumferential Fibrosis of the Ascending Aorta After COVID Infection.

After recovering from severe COVID-19 infection, 2 women presented with chest pain. Computed tomographic angiography suggested acute ascending aortic dissection. At operation in both patients, the ascending aorta was encased in dense fibrous tissue, within which were focal collections of mononuclear cells, including many plasma cells. There was no entry tear or dissection. Such findings we have not encountered previously, and PubMed search of "periaortic fibrosis and COVID-19" yielded no similar cases or possible relation.

Aortic Intimal-Medial Tear Without Dissection, the Marfan Syndrome, and Its Forme Fruste Variety.

Acute aortic dissection is a relatively common disease involving the aorta. All aortic dissections start with an intimal-medial tear prior to the medial dissection. Several cases of aortic intimal-medial tear without dissection have been reported previously, but only one article presented a photograph of the intimal-medial tear. Herein, we describe 16 patients whose ascending aortas were operatively excised because of what clinically was believed to be acute aortic dissection. Of the 16 patients, 14 had aortic intimal-medial tears without dissection and the other 2 had acute medial dissection of the aorta adjacent to a healed aortic intimal-medial tear without dissection. These aortic intimal-medial tears have been seen in the Marfan syndrome, but none of our 16 patients had the Marfan syndrome. At least 9 of the 16 patients, however, had had aortas similar to those seen in the Marfan syndrome (forme fruste variety). Although the 8 surgeons who operated on these 16 patients described the intimal-medial tears as "aortic dissection", only 2 had acute dissection adjacent to a healed intimal-medial tear without dissection. In conclusion, although the aortic intimal-medial tear is the initiator of aortic dissection, some patients with intimal-medial tears have no accompanying dissection.

Diagnosing aortic syphilis.

Described herein is a morbidly obese 57-year-old man with an aneurysm involving the tubular portion of the aorta. Examination of the wall of the operatively resected aneurysm disclosed classic findings of aortic syphilis, a condition that clearly has not disappeared. If there is an aneurysm involving the tubular portion of the ascending aorta in the absence of aortic dissection or involvement of the sinuses of Valsalva, the most likely diagnosis is aortic syphilis. In these circumstances, the serologic test for syphilis is often negative.

Ventricular Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy (Analysis Spanning 60 Years Of Practice): AJC Expert Panel.

Surgical myectomy remains the time-honored primary treatment for hypertrophic cardiomyopathy patients with drug refractory limiting symptoms due to LV outflow obstruction. Based on >50 years experience, surgery reliably reverses disabling heart failure by permanently abolishing mechanical outflow impedance and mitral regurgitation, with normalization of LV pressures and preserved systolic function. A consortium of 10 international currently active myectomy centers report about 11,000 operations, increasing significantly in number over the most recent 15 years. Performed in experienced multidisciplinary institutions, perioperative mortality for myectomy has declined to 0.6%, becoming one of the safest currently performed open-heart procedures. Extended myectomy relieves symptoms in >90% of patients by ≥ 1 NYHA functional class, returning most to normal daily activity, and also with a long-term survival benefit; concomitant Cox-Maze procedure can reduce the number of atrial fibrillation episodes. Surgery, preferably performed in high volume clinical environments, continues to flourish as a guideline-based and preferred high benefit: low treatment risk option for adults and children with drug refractory disabling symptoms from obstruction, despite prior challenges: higher operative mortality/skepticism in 1960s/1970s; dual-chamber pacing in 1990s, alcohol ablation in 2000s, and now introduction of novel negative inotropic drugs potentially useful for symptom management.

Fatal mitral stenosis secondary to massive mitral annular calcium.

Described herein are two patients who developed fatal acute heart failure due to mitral stenosis (MS) secondary to massive mitral annular calcium (MAC). This mechanism causing MS is rather unusual, but, nevertheless, it is probably the most common cause of MS today in Native Americans. Operative intervention for MS secondary to MAC is, as shown previously, hazardous and too frequently fatal.

Body Mass Index in Patients With Operatively-Excised Congenitally Bicuspid Aortic Valves Comparing Those With Stenotic to Those With Purely Regurgitant Valves.

Several publications have examined the body mass index (BMI) in patients having aortic valve replacement for aortic stenosis with and without a congenitally bicuspid aortic valve (BAV). The present study examines BMI in 536 adults having aortic valve replacement for a congenitally BAV and compares the BMI in those with stenotic vs those with purely regurgitant BAVs. No significant differences were found between the 2 groups.