Carcinoid Heart Disease in Association with a Primary Ovarian Carcinoid Tumor: Diagnostic Role of Echocardiography

We describe a patient with carcinoid heart disease secondary to a primary ovarian carcinoid tumor who initially presented with unexplained right-sided heart failure. The patient did not exhibit any of the typical clinical manifestations of the carcinoid syndrome. Echocardiography demonstrated typical features of carcinoid heart disease and played an important role in the detection and management of a condition that was previously unrecognized.     

Benefits and Limitations of Multimodality Imaging in the Diagnosis of a Primary Cardiac Lymphoma

Primary cardiac tumors are far rarer than tumors metastatic to the heart. Angiosarcoma is the primary cardiac neoplasm most frequently detected; lymphomas constitute only 1% of primary cardiac tumors.We present the case of a 55-year-old woman with a recently diagnosed intracardiac mass who was referred to our institution for consideration of urgent orthotopic heart transplantation. Initial images suggested an angiosarcoma; however, a biopsy specimen of the mass was diagnostic for diffuse large B-cell lymphoma. The patient underwent chemotherapy rather than surgery, and she was asymptomatic 34 months later.We use our patient''s case to discuss the benefits and limitations of multiple imaging methods in the evaluation of cardiac masses. Certain features revealed by computed tomography, cardiac magnetic resonance, and positron emission tomography can suggest a diagnosis of angiosarcoma rather than lymphoma. Cardiac magnetic resonance and positron emission tomography enable reliable distinction between benign and malignant tumors; however, the characteristics of different malignant tumors can overlap. Despite the great usefulness of multiple imaging methods for timely diagnosis, defining the extent of spread and the hemodynamic impact, and monitoring responses to treatment, we think that biopsy analysis is still warranted in order to obtain a correct histologic diagnosis in cases of suspected malignant cardiac tumors.     

Recurrent Primary Cardiac Lymphoma on Aortic Valve Allograft: Implications for Therapy

Primary malignant cardiac lymphomas associated with grafts are extremely rare: to our knowledge, only 6 cases of prosthesis-associated B-cell lymphoma have been reported. Ours is the first report of recurrent diffuse large B-cell lymphoma associated with aortic valve allografts.We treated a 60-year-old man who presented in early 2007 with aortic valve endocarditis. He underwent aortic valve replacement with an allograft; the resected native valve showed active endocarditis without tumor. In January 2011, the patient underwent repeat aortic valve replacement because of symptomatic aortic regurgitation. The explanted valve specimen displayed diffuse large B-cell lymphoma. In September 2011, the patient presented with fever and a mass around the aortic valve. He died in January 2012. On autopsy, the explanted replacement valve displayed recurrent diffuse large B-cell lymphoma. The recurrent lymphoma on a new graft leads us to believe that this tumor is more aggressive than had been thought. We propose early systemic chemotherapy, in addition to tumor resection, for the possibility of a better prognosis. We discuss our patient''s case and review the relevant medical literature.     

Papillary Fibroelastoma of the Aortic Valve: Analysis of 21 Cases,Including a Presentation with Cardiac Arrest

Cardiac papillary fibroelastoma is a rare, benign tumor, arising predominantly from cardiac valves. This tumor can cause a variety of symptoms due to thromboembolism. We describe our single-center surgical experience with papillary fibroelastoma of the aortic valve.From April 2004 through June 2013, 6,530 patients underwent cardiac surgery. Of those, 6,098 patients were included in the final analysis. Twenty-one patients (0.34%) underwent surgical resection of 30 papillary fibroelastomas of the aortic valve.Most patients (67%) were incidentally diagnosed to have cardiac papillary fibroelastoma. The usual symptom was cerebral infarction (in 5 of 7 symptomatic patients). A rare presentation of papillary fibroelastoma in one patient was cardiac arrest caused by left main coronary artery ostial obstruction. Tumor size was not related to patient age (Pearson correlation coefficient, 0.34; P=0.13). Neither the number of tumors (1.43 ± 0.72 vs 1.43 ± 0.62) nor tumor size (8.14 ± 2.42 vs 8.07 ± 3.31 mm) was significantly different between symptomatic and asymptomatic patients. All lesions were resected by means of the simple shave technique. There were no operative or 30-day deaths. Follow-up echocardiograms showed no tumor recurrence (mean follow-up duration, 17 ± 14 mo).We identified no significant relationship among tumor size, number of tumors, symptoms, or patient age. Because simple shave excision of the tumor can be safely achieved without evidence of tumor recurrence, we conclude that surgical resection can be reasonable in asymptomatic patients.     

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.     

Transvenous Transcatheter Valve-in-Valve Implantation after Bioprosthetic Tricuspid Valve Failure

We describe the case of a 38-year-old man with a history of metastatic testicular cancer who had undergone multiple thoracic surgical procedures, including tricuspid valve replacement with a bioprosthetic valve as a result of tricuspid involvement of his malignancy. He presented at our outpatient cardiology clinic with worsening fatigue, shortness of breath, and peripheral edema, investigation of which revealed severe tricuspid bioprosthesis stenosis with central regurgitation. Because of the patient''s medical history, he was considered to be a high-risk surgical candidate. Therefore, transcatheter tricuspid valve-in-valve implantation of a 26-mm Edwards Sapien^® valve was attempted through a transjugular approach. The procedure restored tricuspid valvar competence and substantially improved the patient''s symptoms. We discuss the technical aspects of this case and briefly review the usefulness of the valve-in-valve technique in the tricuspid position.     

Left Atrial Myxoma Causing Coronary Steal: An Atypical Cause of Angina

Cardiac myxomas are rare primary cardiac tumors that usually present with dyspnea or manifestations of systemic embolization. Coronary steal is a rare phenomenon of unbalanced blood flow that is seen primarily in patients who have undergone coronary artery bypass grafting and have subclavian artery stenosis.We report the case of a 72-year-old woman who presented with fatigue, weakness, and exertional chest heaviness and had abnormal results on a cardiac stress test. The results of coronary angiography showed no obstructive coronary artery disease but revealed a large intracardiac left atrial mass that was supplied by 2 anomalous coronary arteries. The patient underwent successful ligation of the anomalous coronary arteries and resection of the mass, which was histologically an atrial myxoma. The patient''s symptoms resolved, and results of a repeat cardiac stress test were normal.To our knowledge, this is the first report of a highly vascularized atrial myxoma that caused coronary steal with objective evidence of ischemia, and with subsequent resolution after resection of the mass and ligation of the anomalous coronary arteries.     

Use of a Sandwich Technique to Repair a Left Ventricular Rupture after Mitral Valve Replacement

One difficulty with external repair of left ventricular rupture after mitral valve replacement is collateral bleeding in friable myocardium adjacent to the rupture. The bleeding is caused by tension on the closing sutures, whether or not pledgets have been used.We report the case of a 69-year-old woman who underwent an uneventful mitral valve replacement. After cardiopulmonary bypass was terminated, brisk bleeding started from high in the posterior left ventricular wall, typical of a type III defect. We undertook external repair, placing a plug of Teflon felt into the cavity of the rupture and sandwiching it into place with pledgeted mattress and figure-of-8 sutures. The space occupied by the plug decreased the distance needed to obliterate the defect and thereby reduced the tension on the sutures necessary to achieve hemostasis. This simple technique enabled closure of the defect and avoided collateral tears that would have compromised an otherwise successful repair. Two years postoperatively, the patient had normal mitral valve function and no left ventricular aneurysm. In addition to reporting the patient''s case, we review the types of left ventricular rupture that can occur during mitral valve replacement and discuss the various repair options.     

Concurrent Aortic Valvular Disease and Pulmonary Sequestration: Clinical Implications

Pulmonary sequestration refers to segmental lung tissue that has no connection with the bronchial tree or pulmonary arteries. In adults, the clinical sequelae are usually related to infection. Patients are typically referred for sequestrectomy even when they are asymptomatic. There are no guidelines for treating patients who have pulmonary sequestration and coexisting cardiac valvular disease, in which case the venous drainage patterns of sequestra pose the additional risks of infective endocarditis and volume overload.We present the cases of 2 adult patients—one symptomatic and one asymptomatic—who had concurrent aortic valvular disease and pulmonary sequestration, and we discuss the factors involved in our evaluation of their cardiac risk and our treatment decisions. In view of the sparse data to predict cardiac risks, we think that pulmonary sequestrectomy in adult patients with concurrent valvular conditions should be considered on a case-by-case basis.     

Benign Metastasizing Leiomyoma in the Heart of a 45-Year-Old Woman

We report a rare case of benign metastasizing leiomyoma in the heart of a 45-year-old woman 2 years after a uterine leiomyoma had been discovered during hysterectomy. Computed tomograms at presentation showed a large mixed cystic mass in the pelvis and bilateral lung nodules suggestive of metastatic disease. A large cardiac mass, attached to the chordae of the tricuspid valve and later shown to be histopathologically consistent with uterine leiomyoma, was successfully resected through a right atriotomy. This case suggests that benign metastasizing leiomyoma should be considered in the differential diagnosis of right-sided cardiac tumors.     

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.     

Valve Replacement with a Sutureless Aortic Prosthesis in a Patient with Concomitant Mitral Valve Disease and Severe Aortic Root Calcification

Aortic valve replacement with concomitant mitral valve surgery in the presence of severe aortic root calcification is technically difficult, with long cardiopulmonary bypass and aortic cross-clamp times.We performed sutureless aortic valve replacement and mitral valve annuloplasty in a 68-year-old man who had severe aortic stenosis and moderate-to-severe mitral regurgitation. Intraoperatively, we found severe calcification of the aortic root. We approached the aortic valve through a transverse aortotomy, performed in a higher position than usual, and we replaced the valve with a Sorin Perceval S sutureless prosthesis. In addition, we performed mitral annuloplasty with use of an open rigid ring.The aortic cross-clamp time was 63 minutes, and the cardiopulmonary bypass time was 83 minutes. No paravalvular leakage of the aortic prosthesis was detected 30 days postoperatively.Our case shows that the Perceval S sutureless bioprosthesis can be safely implanted in patients with aortic root calcification, even when mitral valve disease needs surgical correction.     

Use of a Melody Pulmonary Valve in Transcatheter Valve-in-Valve Replacement for Tricuspid Valve Bioprosthesis Degeneration

Bioprosthetic heart valves can degenerate and fail over time. Repeat surgery as a means of replacement increases morbidity and mortality rates, and some patients are not candidates for reoperation. A newer treatment, percutaneous transcatheter valve-in-valve implantation, might delay or substitute for invasive procedures. We present the case of a 51-year-old woman, a poor candidate for surgery who had prosthetic tricuspid valve degeneration and stenosis. We successfully performed valve-in-valve placement of a Melody^® valve, using a procedure originally intended to treat pulmonary valve conduit obstruction or regurgitation. To our knowledge, this is among the first case reports to describe the use of the Melody pulmonary valve in transcatheter valve-in-valve replacement for prosthetic tricuspid stenosis that was otherwise not correctable. Additional data and longer follow-up periods are necessary to gain an understanding of ideal indications and selection of patients for the percutaneous transcatheter treatment of tricuspid valve stenosis.     

Complete Atrioventricular Septal Defect and Pulmonary Stenosis Diagnosed in a 49-Year-Old Woman after 10 Uneventful Births

Atrioventricular septal defects constitute 4% of all congenital cardiac malformations. Patients with complete atrioventricular septal defect rarely survive for decades without surgical treatment. Pulmonary stenosis can provide a delicate balance between the pulmonary and systemic circulations and thereby increase longevity. We present the case of a 49-year-old woman whose complete atrioventricular septal defect and associated pulmonary stenosis were diagnosed only after she had given birth to 10 live children through uneventful spontaneous delivery. We discuss her successful surgical treatment in terms of the available medical literature.     

Ochronotic Involvement of the Aortic and Mitral Valves in a 72-Year-Old Man

Ochronosis, an autosomal recessive metabolic disorder, causes an excess of homogentisic acid that results in adverse pigmentation, calcification, and inflammation of cartilaginous and other tissues. Cardiovascular abnormalities are less frequently reported than are other manifestations. In rare cases, ochronosis can cause valvular heart disease. We report the case of a 72-year-old man with aortic stenosis and mitral insufficiency who was diagnosed with ochronosis while undergoing surgical aortic and mitral valve replacement. We discuss the history and surgical management of alkaptonuric ochronosis.     

Cardiac Papillary Fibroelastoma: Single-Institution Experience with 14 Surgical Patients

In general, treatment for symptomatic and asymptomatic cardiac papillary fibroelastoma is surgical resection—particularly of left-sided lesions, because of the risk of systemic embolization. However, few institutions have enough experience with these tumors to validate this approach. We present our institutional experience with papillary fibroelastoma and discuss our current approach.We searched our institution''s cardiac tumor database, identified all patients diagnosed with cardiac papillary fibroelastoma from 1992 through 2014, and recorded the clinical and pathologic characteristics of each case. We found 14 patients (mean age, 60.5 ± 12.3 yr) who had 18 lesions. Eleven patients (79%) were symptomatic; however, we could not always definitively associate their symptoms with a cardiac tumor. Most lesions were solitary and ≤1.5 cm in diameter; half involved the left side of the heart. All 18 lesions were surgically excised. There were no operative or 30-day deaths, and no patient needed valve replacement postoperatively. There was one late death; at one year, another 3 patients were lost to follow-up, and the others were alive without tumor recurrence.Because of the embolic risk inherent to intracardiac masses and our relatively good postoperative outcomes, we recommend the surgical resection of all left-sided papillary fibroelastomas in surgical candidates, and we discuss with patients the advisability of resecting right-sided lesions.     

Platypnea-Orthodeoxia Syndrome Caused by an Intracardiac Shunt

Platypnea-orthodeoxia syndrome, a rare condition characterized by posture-related dyspnea, is usually caused by an intracardiac shunt, hepatopulmonary syndrome, or shunting resulting from severe pulmonary disease.We report the case of a 33-year-old woman who presented with increasing dyspnea and oxygen desaturation when she sat up or arose. Our diagnosis was platypnea-orthodeoxia syndrome. A lead of a previously implanted pacemaker exacerbated a severe tricuspid regurgitant jet that was directed toward the patient''s intra-atrial septum. Percutaneous closure of a small secundum atrial septal defect eliminated right-to-left shunting and substantially improved the patient''s functional status. In addition to this case, we discuss this unusual condition.     

Cardiac Sarcoidosis Presenting as Constrictive Pericarditis

In patients with cardiac sarcoidosis, the sarcoid granulomas usually involve the myocardium or endocardium. The disease typically presents as heart failure with ventricular arrhythmias, conduction disturbances, or both. Constrictive pericarditis has rarely been described in patients with sarcoidosis: we found only 2 reports of this association.We report the case of a 57-year-old man who presented with clinical and hemodynamic features of constrictive pericarditis, of unclear cause. He was admitted for treatment of recurrent pleural effusion. After a complicated hospital course, he underwent pericardiectomy. His clinical and hemodynamic conditions improved substantially, and he was discharged from the hospital in good condition. The pathologic findings, the patient''s clinical course, and his response to pericardiectomy led to our diagnosis of cardiac sarcoidosis presenting as constrictive pericarditis. In addition to the patient''s case, we discuss the nature and diagnostic challenges of cardiac sarcoidosis. Increased awareness of this disease is necessary for its early detection, appropriate management, and potential cure.     

Alternative Surgical Approach to Repairing a Giant Sinus of Valsalva Aneurysm

We report our method of surgically repairing an unruptured giant aneurysm of the right coronary sinus of Valsalva, a lesion that caused moderate aortic regurgitation but no symptoms in a 61-year-old woman. We excised the aneurysm, reconstructed the right sinus of Valsalva with use of a patch, performed mechanical aortic valve replacement directly through the excised aneurysm''s cavity, and constructed a single bypass graft to the right coronary artery. The patient was discharged from the hospital after 5 days. Twelve months postoperatively, her clinical and echocardiographic results were normal, and she was doing well. To our knowledge, our surgical approach to this repair has not been described previously.     

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.