Down Syndrome with Complete Atrioventricular Septal Defect,Hypertrophic Cardiomyopathy,and Pulmonary Vein Stenosis

The prevalence of congenital heart disease in infants with Down syndrome is 40%, compared with 0.3% in children who have normal chromosomes. Atrioventricular and ventricular septal defects are often associated with chromosomal aberrations, such as in trisomy 21, whereas hypertrophic cardiomyopathy is chiefly thought to be secondary to specific gene mutations. We found only one reported case of congenital hypertrophic cardiomyopathy and atrioventricular septal defect in an infant with Down syndrome. Here, we report atrioventricular septal defect, hypertrophic cardiomyopathy, and pulmonary vein stenosis in a neonate with Down syndrome—an apparently unique combination. In addition, we discuss the relevant medical literature.     

Cor Triatriatum Dexter and Atrial Septal Defect in a 43-Year-Old Woman

Cor triatriatum dexter is a rare congenital heart anomaly in which a membrane divides the right atrium into 2 chambers. We report the case of a 43-year-old woman who had cor triatriatum dexter and a large atrial septal defect. During attempted percutaneous closure, the balloon disrupted the membrane and revealed that the defect had no inferior rim, precluding secure placement of an Amplatzer Septal Occluder. Surgical treatment subsequently proved to be successful.In patients with an incomplete membrane and a septal defect with well-defined rims, percutaneous treatment can be the first choice. In patients who have cor triatriatum dexter and unfavorable anatomic features or concomitant complex heart anomalies, open-heart surgery remains the gold standard for treatment.     

Emergent Surgical Pulmonary Embolectomy in a Pregnant Woman: Case Report and Literature Review

Acute pulmonary embolism is a leading cause of death during pregnancy and delivery in the United States. We describe the case of a 25-year-old woman who presented in car-diogenic shock in week 38 of her first pregnancy. After the emergent cesarean delivery of a healthy male neonate, the mother underwent immediate surgical pulmonary embolec-tomy. We confirmed the diagnosis of pulmonary embolism intraoperatively by means of transesophageal echocardiography and removed large clots from the patient''s pulmonary arteries. Mother and child were doing well, 27 months later. In addition to presenting our patient''s case, we discuss the other relevant reports and the options for treating massive pulmonary embolism during pregnancy.     

Trousseau Syndrome in a 25-Year-Old Woman with Occult Colon Malignancy,Lynch Syndrome,and Chronic Thromboembolic Pulmonary Hypertension

We present a rare case of thrombosis associated with an occult colon malignancy (Trousseau syndrome) in a 25-year-old woman who also presented with previously unidentified Lynch syndrome and acute-on-chronic thromboembolic pulmonary hypertension. Staged treatment included bilateral pulmonary endarterectomy under deep hypothermic circulatory arrest, followed 11 days later by laparoscopic subtotal colectomy and creation of a primary anastomosis. The patient tolerated both procedures well and recovered normal functional status. Final pathologic analysis of the resected colon mass revealed a pT3N0, stage IIA adenocarcinoma; no adjuvant therapy was administered. At her one-year follow-up visit, the patient was cancer-free, remained on lifelong apixaban anticoagulation, and was undergoing routine monitoring and genetic counseling. This case highlights the need for multidisciplinary management of a patient with severe chronic thromboembolic pulmonary hypertension and a concomitant malignancy.     

Late Postoperative Prosthetic Pulmonary Valve Endocarditis in a 13-Year-Old Girl with Repaired Tetralogy of Fallot

Prosthetic pulmonary valve endocarditis has infrequently been described outside large cohort reviews, which have typically focused on infections of the left-sided heart valves. Hence, the pathogenesis, clinical presentation, and management principles of prosthetic pulmonary valve endocarditis have not been well differentiated from those of infected aortic and mitral valves. More patients with repaired tetralogy of Fallot are reaching adulthood and will need pulmonary valve implantation. Consequently, a focus on this infrequent but serious cardiac infection is needed, to learn what characteristics might distinguish it from infections of left-sided heart valves.We report the case of a 13-year-old girl with repaired tetralogy of Fallot who presented with fever and nonspecific symptoms. The patient initially failed to meet the Duke criteria for endocarditis but was then found to have endocarditis of her prosthetic pulmonary valve. We explanted the valve and replaced it with a pulmonary homograft, after which the patient had no infectious sequelae. In addition to presenting the patient''s case, we review the literature on surgically inserted prosthetic pulmonary valves and discuss the primary management concerns when those valves become infected with endocarditis.     

Pulmonary Hemodynamics and Outcome in a Large Cohort of Patients with Sinus Venosus Septal Defect

Background: Left-to-right shunt in sinus venosus septal defect (SVSD) may affect resistive (pulmonary vascular resistance–PVR) and elastic (pulmonary artery compliance-PAC) pulmonary artery properties. This study aimed at evaluating (1) impact of age, (2) pulmonary hemodynamics, and (3) outcome in a large cohort of SVSD patients. Methods: This study included 136 patients with SVSD (median age at diagnosis 14 (IQR 5–48) years, 47% male) of which 87 underwent catheterization. Pressures were measured and cardiac output was evaluated using the Fick principle at diagnosis. PVR, PAC and their product (RC time) were calculated. Results: Surgical repair was performed in 128 (94%) at a median age of 13 (IQR 5– 43) years. During a median follow-up time of 31 (IQR 17–55) years, 12 (9%) patients died, 13 (10%) developed heart failure, 4 (3%) Eisenmenger syndrome, 19 (14%) atrial arrhythmia, 6 (4%) sick sinus syndrome and 7 (5%) required pacemaker implantation. In those who underwent catheterization, median shunt ratio was 2.5 (IQR 2.0–2.9). Thirty (34%) had mean PA pressure ≥25 mmHg. PVR indexed, PAC indexed, and RC time was 3.5 (IQR 2.4–7.5) WU.m², 1.8 (IQR 1.3–2.5) mL/mmHg.m² and 0.39 (0.26–0.53) sec with an inverse hyperbolic relationship between PVR and PAC. Mean PA pressure (P < 0.0001); wedge pressure (P = 0.001), PVR indexed (P = 0.002) and PAC indexed (P = 0.002) changed significantly with age at diagnosis, but shunt ratio did not. Conclusion: SVSD has good long-term outcome, albeit with late morbidities. Thirty-four percent has mean PA pressure ≥25 mmHg, but Eisenmenger syndrome is rare (3%). PVR and PAC are inversely related and change significantly with older age.     

Complete Atrioventricular Septal Defect with Truncus Arteriosus and Interrupted Aortic Arch in the Fetus

Complete atrioventricular septal defect is generally diagnosed on the four‐chamber view of the fetal heart. This defect can be accompanied by additional outflow tract lesions, including truncus arteriosus. Although truncus arteriosus in isolation can be associated with interrupted aortic arch, we describe a fetal case with the rare association of all three diagnoses: complete atrioventricular septal defect with truncus arteriosus and interrupted aortic arch. This case points to the importance of evaluating the complete fetal heart even after one congenital cardiac abnormality is noted. The complexity of heart disease has implications for prenatal counseling and postnatal management.     

Silent Kawasaki Disease Affecting Multiple Coronary Arteries in a 39-Year-Old Egyptian Woman

Kawasaki disease, an acute febrile illness, can cause vasculitis in the coronary arteries. It is the chief acquired cause of myocardial infarction and sudden cardiac death in infants, children, and young adults in developed countries. We report a case of chronic, silent Kawasaki disease complicated by multivessel thrombosis in a 39-year-old Egyptian woman. The patient presented with progressive, unstable angina but was otherwise asymptomatic and at negligible risk of ischemic heart disease. Coronary angiograms showed critical arterial stenosis with multiple aneurysms. During revascularization surgery, the patient''s harvested left internal mammary artery was found to have occlusive lesions and aneurysmal areas that made it unfit for bypass grafting, and subsequent histopathologic examination revealed features characteristic of chronic Kawasaki disease–associated systemic vasculitis.We think that this is only the second report of Kawasaki disease in the Arabian Mediterranean region. In addition to the patient''s case, we discuss the epidemiology and management of Kawasaki disease, in hopes of increasing clinicians'' awareness.     

Heavy Lifting Causing Spontaneous Coronary Artery Dissection with Anterior Myocardial Infarction in a 54-Year-Old Woman

Spontaneous coronary artery dissection in association with strenuous exercise and weightlifting is rather sparsely described in the medical literature. Diagnosis and treatment of this rare condition is a challenge, but prompt recognition and appropriate early choice of angioplasty or surgery can lead to a good outcome.We report the case of a postmenopausal 54-year-old woman who presented with anterior myocardial infarction caused by spontaneous dissection of the left anterior descending coronary artery after she had lifted a heavy weight while gardening. The patient was treated successfully by means of angioplasty and the implantation of 3 drug-eluting stents. In addition to presenting the patient''s case, we review the topical medical literature.     

Massive Pulmonary Artery Aneurysm Causing Left Main Coronary Artery Compression in the Absence of Pulmonary Hypertension

We report the case of a 62-year-old woman who presented with classic symptoms of stable angina. Cardiac images and catheterization results revealed absent pulmonary valve syndrome and compression of the left main coronary artery by a massively dilated pulmonary artery aneurysm. The patient''s anginal symptoms were relieved after pulmonary arterioplasty.Others have described proximal left main coronary artery compression in the presence of a dilated and hypertensive pulmonary artery. To our knowledge, this is the first case in which a pulmonary artery aneurysm caused left main coronary insufficiency in the absence of pulmonary hypertension—a clinically important complication of congenital pulmonary valve-related pulmonary arteriopathy.     

Percutaneous Repair of Post-Myocardial Infarction Ventricular Septal Defect: Current Approaches and Future Perspectives

Post-myocardial infarction ventricular septal defect is a devastating complication of ST-elevation myocardial infarction. Although surgical intervention is considered the gold standard for treatment, it carries high morbidity and mortality rates. We present 2 cases that illustrate the application of percutaneous closure of a post-myocardial infarction ventricular septal defect: the first in a patient who had undergone prior surgical closure and then developed a new shunt, and the second as a bridge to definitive surgery in a critically ill patient.     

Late Bacterial Endocarditis in an Intravenous Drug User With an Amplatzer Septal Occluder

Infective endocarditis of a fully endothelialized cardiac prosthesis, and especially the late presentation of endocarditis, challenges our current understanding of device-related complications. Late bacterial endocarditis associated with the Amplatzer Septal Occluder, a device frequently used to close atrial septal defects, has been documented only rarely. We report the case of an intravenous drug user who had late infective endocarditis associated with his Amplatzer Septal Occluder, secondary to methicillin-sensitive Staphylococcus aureus bacteremia nearly 14 years after device insertion. The patient recovered after surgical excision and débridement of the vegetative mass, which may be the first time that a surgical approach has been taken to treat this condition. This report corroborates the need for late screening of high-risk patients who have septal occluder devices.     

Cheyne-Stokes Respiration in a 17-Year-Old Boy Awaiting Heart Transplantation

Cheyne-Stokes respiration is a pattern of alternating central apnea and hyperpnea. It is well described in adults with congestive heart failure, but not in children.We report the case of a 17-year-old boy whose systolic heart failure was complicated by Cheyne-Stokes respiration. He was given supportive therapy until heart transplant, after which his Cheyne-Stokes respiration clinically resolved. Clinicians should be aware of this uncommon condition in pediatric and adolescent patients who have advanced heart failure and irregular breathing.     

Embolic Stroke Caused by Staphylococcus lugdunensis Endocarditis Complicating Vasectomy in a 36-Year-Old Man

Staphylococcus lugdunensis is part of the native flora in the inguinal region of the body. Inguinal surgeries, such as vasectomy, place carriers of this aggressive pathogen at risk for contamination. Native-valve endocarditis caused by coagulase-negative S. lugdunensis has a rapid and complicated clinical course. The pathogenicity of this organism is not limited to cardiac valvular destruction. We report the case of a 36-year-old man who presented with S. lugdunensis endocarditis, dysarthria, and hemiparesis 5 weeks after a vasectomy. To our knowledge, this is the first report of embolic stroke caused by S. lugdunensis endocarditis. In addition, we discuss the relevant medical literature.     

Resolution of Right-Sided Heart Failure Symptoms after Resection of a Primary Ovarian Carcinoid Tumor

Carcinoid tumors are rare neuroendocrine malignancies that typically originate from the gastrointestinal tract. Patients who are diagnosed with carcinoid heart disease generally have poor prognoses because of advanced metastases during staging and few therapeutic options. We present the case of a 61-year-old woman with right-sided heart failure, secondary to carcinoid heart disease caused by a primary ovarian carcinoid tumor. After undergoing surgical resection of the left ovary and fallopian tube, the patient experienced complete resolution of her heart failure symptoms. In addition to the patient''s case, we discuss the diagnosis, nature, and treatment of this rare condition.     

Cardiomyopathy Induced by Pulmonary Sequestration in a 50-Year-Old Man

A 50-year-old black man presented at the emergency department with midsternal, nonradiating chest pressure and chronic dyspnea on exertion. Four years before the current admission, he had been diagnosed with nonischemic cardiomyopathy at another facility. After our complete evaluation, we suspected that his symptoms arose from left-to-left shunting in association with pulmonary sequestration, a congenital malformation. Our preliminary diagnosis of secondary dilated cardiomyopathy was confirmed by normalization of the patient''s ventricular size and function after lobectomy. To our knowledge, this patient is the oldest on record to present with cardiomyopathy consequent to pulmonary sequestration. His case is highly unusual because of his age and the rapid resolution of his symptoms after lobectomy. We believe that pulmonary sequestration should be included in the differential diagnosis of dilated cardiomyopathy.     

Acute Coronary Thrombosis and Multiple Coronary Aneurysms in a 22-Year-Old Man with the Human Immunodeficiency Virus

The human immunodeficiency virus (HIV) can cause diverse cardiovascular complications. In HIV patients on antiretroviral therapy, the prevalence of myocardial infarction has steadily increased over the years. Young patients who are naïve to antiretroviral therapy and who experience coronary events are not well represented in the medical literature. We describe the case of a 22-year-old man, infected with HIV for 4 years and never treated with antiretroviral therapy, who emergently presented with a non-ST-segment-elevation myocardial infarction. Coronary angiograms revealed thrombosis and multiple coronary artery aneurysms; however, no areas of atherosclerotic stenosis were apparent. He was successfully treated with coronary stenting, antiplatelet therapy, and anticoagulation. Nine months after the initial presentation, he exhibited excellent exercise capacity, and no ischemia was evident. We discuss the various therapeutic approaches in this case.     

Neocuspidization of the Pulmonary Valve with Autologous Pericardium in the Adult Patient with Ventricular Septal Defect and Infective Endocarditis: A Case Report and Review of the Literature

Congenital heart disease (CHD) is one of the risk factors for developing infective endocarditis (IE). Right-sided IE occurs in 5%–10% of endocarditis cases, and pulmonary valve (PV) is involved in less than 2% of such patients. Literature data are few, and optimal treatment methods, indications for surgery, and types of operative techniques are still under debate. We present an adult patient with a rare combination of the ventricular septal defect (VSD) and PV IE who underwent surgical treatment. Neocuspidization with autologous pericardium was utilized for the reconstruction of his PV. We discuss details of this novel surgical technique.     

Large Patent Ductus Arteriosus in a 44-Year-Old Woman Leading to Calcium Deposition in the Left Atrium and Mitral and Aortic Valves

This report describes unusual autopsy findings in a 44-year-old woman who had a large, calcified patent ductus arteriosus that produced substantial left-to-right shunting. The patient died in 1962, 7 days after patch closure of the aortic orifice of the ductus. Numerous calcific deposits were present in the mural left atrial endocardium, the mitral valve leaflets and annulus, and the aortic valve cusps. The cause of the left-sided calcific deposits was perhaps related to the patient''s several-decades-old giant aortopulmonary shunt, causing a major increase in the volume of blood passing through the left-sided cardiac chambers in comparison with the volume in the right side. To our knowledge, such findings in a patient with patent ductus arteriosus have not been reported previously.