Aortic Valve Reconstruction with Use of Pericardial Leaflets in Adults with Bicuspid Aortic Valve Disease: Early and Midterm Outcomes

In this study, we retrospectively analyzed the outcomes of adults with bicuspid aortic valve (BAV) disease who underwent aortic valve reconstructive surgery (AVRS), consisting of replacement of the diseased BAV with 2 or 3 pericardial leaflets plus fixation of the sinotubular junction for accurate and constant leaflet coaptation. From December 2007 through April 2013, 135 consecutive patients (mean age, 49.2 ± 13.1 yr; 73.3% men) with symptomatic BAV disease underwent AVRS. Raphe was observed in 84 patients (62.2%), and the remaining 51 patients had pure BAV without raphe. A total of 122 patients (90.4%) underwent 3-leaflet reconstruction, and 13 (9.6%) underwent 2-leaflet reconstruction. Concomitant aortic wrapping with an artificial graft was performed in 63 patients (46.7%).There were no in-hospital deaths and 2 late deaths (1.5%); 6 patients (4.4%) needed valve-related reoperation. The 5-year cumulative survival rate was 98% ± 1.5%, and freedom from valve-related reoperation at 5 years was 92.7% ± 3.6%. In the last available echocardiograms, aortic regurgitation was absent or trivial in 116 patients (85.9%), mild in 16 (11.9%), moderate in 2 (1.5%), and severe in one (0.7%). The mean aortic valve gradient was 10.2 ± 4.5 mmHg, and the mean aortic valve orifice area index was 1.3 ± 0.3 cm²/m². The 3-leaflet technique resulted in lower valve gradients and greater valve areas than did the 2-leaflet technique. Thus, in patients with BAV, AVRS yielded satisfactory early and midterm results with low mortality rates and low reoperation risk after the initial procedure.     

Papillary Fibroelastoma Incidentally Found on Left Atrial Wall During Minimally Invasive Aortic Valve Replacement

A 60-year-old man was about to undergo minimally invasive aortic valve replacement when transesophageal echocardiography revealed an intracardiac mass on the left atrial free wall. Multimodal images from 5 months earlier had shown no mass. We converted the procedure to open surgery. The excised mass resembled a cardiac myxoma but was determined to be a papillary fibroelastoma. This case illustrates that papillary fibroelastomas can form and grow rapidly, warranting alertness for their unexpected discovery before and during cardiac surgical procedures.     

Cone Reconstruction of Atypical Ebstein Anomaly Associated with Right Ventricular Apical Hypoplasia

Cone reconstruction for tricuspid valve repair has revolutionized the surgical treatment of Ebstein anomaly. We present the case of a 58-year-old woman with atypical Ebstein anomaly and right ventricular apical hypoplasia who was spared from palliative shunt physiology by our use of cone reconstruction.Compared with other techniques, cone reconstruction of the tricuspid valve more closely replicates normal valvular anatomy and function. This surgical procedure can be applied to many anatomic variations of Ebstein anomaly, as in our patient''s apparently unique instance of atypical Ebstein anomaly with right ventricular apical hypoplasia.     

Repair of Congenital Right Atrial Aneurysm Associated With Wolff-Parkinson-White Syndrome in a 5-Year-Old Girl

This report describes our experience with a 5-year-old girl who had an extremely rare presentation of a right atrial aneurysm and associated Wolff-Parkinson-White syndrome. Before being referred to our department, she underwent an ineffective radiofrequency ablation for repeated episodes of paroxysmal supraventricular tachycardia that were causing dizziness, palpitations, and chest discomfort. We resected the aneurysm with good results; she was doing well and was in normal sinus rhythm at the time of her 1-year follow-up visit.     

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.     

Left Ventricular Aneurysm Repair with Use of a Bovine Pericardial Patch

Left ventricular aneurysm, which can impair systolic function, has a reported incidence of 10% to 35% in patients after myocardial infarction. In a 58-year-old woman who had a history of myocardial infarction, we excised a large left ventricular aneurysm and restored left ventricular geometry with use of a bovine pericardial patch. The aneurysm''s characteristics and the patient''s preoperative left ventricular ejection fraction of 0.25 had indicated surgical intervention. The patient had an uneventful postoperative course, and her left ventricular ejection fraction was 0.50 to 0.55 on the 4th postoperative day. This case illustrates the value of surgical treatment for patients who have a debilitating left ventricular aneurysm.     

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.     

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.     

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.     

Endometrial Stromal Sarcoma Metastatic from the Uterus to the Inferior Vena Cava and Right Atrium

Endometrial stromal sarcoma metastases usually occur within the pelvis and rarely involve the great vessels or the heart. We present the case of a 55-year-old woman who was referred for endovascular therapy to treat presumed thrombosis of the inferior vena cava. The suspected thrombus was recalcitrant to endovascular removal with use of an AngioVac venous drainage device. Results of an intraprocedural transvenous biopsy revealed the mass to be the intravascular extension of an endometrial stromal sarcoma. The patient underwent surgical excision of the tumor, and, shortly thereafter, a hysterectomy and salpingo-oophorectomy. This complex case highlights both the rarity of malignancy masquerading as caval thrombus and the importance of multispecialty collaboration.     

Early Detection of a Cavopulmonary Tumor Embolus with the Use of Transesophageal Echocardiography

Pulmonary tumor embolization from renal cell carcinoma is associated with severe cardiopulmonary morbidity and high perioperative mortality rates. We report the case of a 71-year-old woman who presented with right-sided abdominal pain. Magnetic resonance images revealed a mass originating from the upper pole of the right kidney and extending into the infrahepatic portion of the inferior vena cava. Transesophageal echocardiography was continuously used to monitor the mass during intended radical nephrectomy and tumor resection. When the right kidney was mobilized, intracaval thrombus detached and migrated to the patient''s right atrium, causing severe hemodynamic instability. After emergent sternotomy and during the initiation of cardiopulmonary bypass, the mass was no longer echocardiographically detectable in the heart; it was soon removed completely from the left pulmonary artery. The mass was a renal cell carcinoma.We recommend the use of transesophageal echocardiography as an efficient diagnostic tool in the early detection of pulmonary tumor embolization during the resection of renal cell carcinoma that involves the inferior vena cava.     

Use of Oversized Injectable Valves in Growing Children for Total Repair of Right Ventricular Outflow Tract Anomalies (Preliminary Results)

Right ventricular outflow tract surgery was originally confined to transannular patching, in the belief that pulmonary regurgitation was well tolerated. Because follow-up evaluations revealed the deleterious effects of pulmonary regurgitation, surgery today aims to spare or replace the valve. Available replacement devices have short lifetimes, considering growth mismatch in children. We hypothesize that oversizing the right infundibulum anticipates growth and that a squeezed prosthesis can complete the expansion process.The No-React^® Injectable BioPulmonic Valve is designed for right infundibular surgery in adults, and hundreds of implants have shown promising results. We used this device for surgery in babies, with the addition of an innovative oversizing technique. This study evaluates our preliminary results and investigates whether such a technique might reduce growth mismatch.From September 2010 through July 2012, we implanted 11 injectable pulmonic valves. The median age of our patients was 23 months. After opening the right infundibulum, we enlarged it as much as possible with a wide patch. Before completing the patch suture, we injected an oversized valve.No problems occurred during surgery. No major insufficiency or leak was observed. We conclude that prostheses can be quite oversized and perform well even when not completely expanded.Oversized injectable pulmonic valves, shrunken to a smaller diameter, enabled the implantation of a device wider than otherwise possible, without affecting performance. Moreover, the prosthesis tended to return to its original size following growth, thereby reducing growth mismatch. Longer follow-up and larger numbers of patients are needed for verification.     

Successful Use of Surgically Placed Impella 5.0 and Central Extracorporeal Membrane Oxygenation Circuit in a Patient with Postcardiotomy Shock

The Impella 5.0, a percutaneously inserted left ventricular assist device, has been used to support patients who have severe heart failure or who are undergoing high-risk percutaneous coronary intervention. We report our surgical placement of the Impella 5.0, through a graft sewn to the aorta, to unload the left ventricle of a 59-year-old man who was undergoing venoarterial extracorporeal membrane oxygenation for postcardiotomy shock. The patient underwent successful placement of a long-term left ventricular assist device before his discharge from the hospital. The versatility of the Impella 5.0 is exemplified in this patient who was successfully bridged to long-term support.     

Two Cases of Transcatheter Closure of Central Aortopulmonary Shunts: One with an Amplatzer Duct Occluder II and One with an Amplatzer Vascular Plug I

When total correction is not possible in infants who have a cyanotic congenital heart disease, creation of a palliative aortopulmonary shunt is essential. A central aortopulmonary shunt is preferable, because of its technical and hemodynamic advantages. Overcirculation, thrombosis, and stenosis of the shunt are the main postoperative sequelae that necessitate urgent reintervention. Percutaneous transcatheter closure of aortopulmonary shunts can eliminate the need for reoperation and substantially decrease postoperative morbidity and mortality rates. We report our successful transcatheter closures of central aortopulmonary shunts in a 3-month-old infant and a 15-year-old girl, with use of an Amplatzer Duct Occluder II and an Amplatzer Vascular Plug I, respectively. To our knowledge, this is the first report of the transcatheter closure of central aortopulmonary shunts with these 2 devices.     

Improved Systemic Saturation after Ventricular Assist Device Implantation in a Patient with Decompensated Dextro-Transposition of the Great Arteries after the Fontan Procedure

We report the successful implantation of a HeartMate II left ventricular assist device after a failed Fontan procedure in a patient with dextro-transposition of the great arteries. The patient had developed significant intrapulmonary arteriovenous shunting. Despite the theoretical risk of worsening intrapulmonary shunting due to the decrease in systemic vascular resistance after device implantation, our patient did well. He was discharged from the hospital in stable condition and had better oxygen saturation than before the device was implanted. To our knowledge, ours is the 2nd report of the use of a ventricular assist device after the failure of a Fontan procedure, and the first report concerning the effect of ventricular assist device implantation on intrapulmonary shunting.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnoses and Surgical Results in 12 Pediatric Patients

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo–8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.