Intracardiac Lesions Research Articles

Advances in Neonatal Cardiac Surgery

After completing this article, readers should be able to: 1. Describe the developments that have allowed advances in neonatal cardiac surgery. 2. Describe the diagnostic approach to hypoplastic left heart syndrome (HLHS). 3. Delineate the preoperative management of the neonate who has HLHS. 4. Describe the original Norwood procedure and subsequent modifications. 5. Delineate the optimal therapy in the postoperative period for neonates who have a single ventricle. Advances in the surgical and medical management of children who have congenital heart disease (CHD) have led to a dramatic improvement in overall survival and reduced the long-term sequelae of open-heart surgery. With improved survival and decreased morbidity over the past 2 decades, the early complete repair of complex congenital heart problems in preterm and low-birthweight neonates has gained wider acceptance. Cardiologists and cardiothoracic surgeons now consider and perform complete neonatal repair more frequently than palliative surgery in this patient population. The treatment of hypoplastic left heart syndrome (HLHS) best illustrates the evolution of management strategies to improve survival and long-term outcome. A recent modification of the Norwood operation that involves a right ventricle-to-pulmonary artery conduit has led to much improved early postoperative stability and, in many centers, improved survival to a stage II superior cavopulmonary connection. A multidisciplinary team approach is required to provide the necessary care for this complex patient population. In 1938, Robert Gross at Children’s Hospital in Boston successfully ligated a patent ductus arteriosus, and this procedure—performed against the wishes of surgeon-in-chief William Ladd—opened the era of surgery for CHD. (1) Gross and Hufnagel performed detailed animal experiments to develop a technique for treatment of coarctation of the aorta that involves excision and end-to-end anastomosis. (2) Clarence Craaford of Stockholm, Sweden, had visited Gross to observe his experimental work, and in October 1944, Craaford and Nylin were the first to repair aortic …

Presentation and outcome of infective endocarditis in Spain: a retrospective study

To analyze cases of infective endocarditis in patients who attended the Hospital General de Castellón, Spain, between 1999 and 2004. This was a retrospective study. Demographic and clinical characteristics were assessed, variation in presentation over time was analyzed, and factors influencing outcome were determined. A total of 54 cases in 48 patients were included; 33 (61%) were in men. The median patient age was 62 years. Infective endocarditis occurred on a native valve in 36 cases (67%), a mechanical prosthetic valve in 12 (22%), and a pacemaker in six (11%). The mitral valve was the most commonly affected site. Transthoracic and/or transesophageal echocardiography showed a vegetation in 45 (83%) cases, moderate or severe valvular regurgitation in 27 (50%), and intracardiac destructive lesions in five (9%). The outcome in 15 (28%) cases was death, and multivariate analysis disclosed significantly increased risk of death associated with older age, lower serum albumin, and higher white blood cell count. This study confirmed the protean nature of infective endocarditis, and identified several factors predictive of mortality including advanced age, low serum albumin, and high white blood cell count.

Excision After Chemoradiotherapy of Invasive Thymoma Extending into the Right Atrium: Report of a Case

We report a case of invasive thymoma with intracardiac extension, resulting from the progression of intracaval growth, in a 56-year-old woman. Initially, the patient received two courses of chemotherapy, but the tumor showed only a modest response; however, subsequent radiotherapy reduced the tumor size further and the intracardiac lesion disappeared, making it possible to excise the tumor without cardiopulmonary bypass. Thus, when a thymoma does not respond well, we recommend radiotherapy as another treatment option, because its effects may allow for less invasive and more complete tumor excision.

Combined Severe Pectus Excavatum Correction and Aortic Root Replacement in Marfan’s Syndrome

A 23-year-old man with Marfan's syndrome was admitted for repair of annulo-aortic ectasia and severe pectus excavatum. A submammary skin incision approach followed by bilateral subperichondrial resection of abnormal costal cartilages was performed. The left intercostal muscles and perichondrial sheaths were divided 2 inches lateral to the sternum in a parasternal fashion to place the retractor. The aortic root was replaced with a 23-mm St. Jude's composite graft (St. Jude Medical, Inc, St. Paul, MN). Chest wall reconstruction was completed with a high sternal osteotomy and support of the sternum was made with Gortex strips (W.L. Gore & Associates, Inc, Flagstaff, AZ). The patient made an uneventful recovery.

Spectrum of Cardiovascular Disease, Accuracy of Diagnosis, and Outcome in Fetal Heterotaxy Syndrome

Because there is a paucity of information regarding the diagnosis and outcomes of fetal heterotaxy syndrome (HS), this study sought to determine the spectrum of cardiac pathology, accuracy of diagnosis, and outcome of fetal HS. All cases of fetal HS encountered in the investigators' institution over a 10-year period through 2002 were identified. Prenatal and postnatal echocardiograms and medical records were reviewed. Seventy-one fetuses were diagnosed with HS, including 48 with left atrial isomerism (LAI) and 23 with right atrial isomerism (RAI). For LAI and RAI, most intracardiac lesions, the pulmonary venous connections, and superior vena caval anatomy were correctly diagnosed in utero (93%, 86%, and 77% accuracy, respectively), whereas hepatic venous connections and inferior vena caval-atrial connections in RAI were difficult to define (65% and 56% accuracy, respectively). Of 32 continued and followed pregnancies with LAI, 22 are currently alive at 48 +/- 30 months. Heart block and associated major extracardiac pathology were significantly more common in nonsurvivors with fetal LAI (p = 0.007 and 0.024, respectively). Outcomes were even worse for prenatally diagnosed RAI: of 14 continued pregnancies, only 3 are currently alive. In conclusion, fetal HS is associated with a broad spectrum of cardiac pathology, which can be diagnosed accurately in utero. Fetal LAI is associated with a mortality of 31%, with heart block and extracardiac pathology as primary risk factors for perinatal mortality. The outcome of prenatally diagnosed RAI is poor.

Cardiovascular Involvement in 8 Dogs with Blastomyces Dermatitidis Infection

Blastomycosis is a common systemic fungal infection in dogs. Dogs with cardiovascular involvement may have abnormalities in electrical conduction and valvular function, and may have a worse prognosis. Eight client-owned animals. Dogs with cardiovascular lesions caused by blastomycosis were identified from retrospective evaluation of medical records. Five dogs had de novo infections and 3 had recurrences of previously treated infections. Harsh labored breathing, lethargy, and anorexia were the most common historic complaints. Three dogs had syncope. Physical examination and clinicopathologic data were typical of blastomycosis and included dyspnea, increased lung sounds, and lethargy. In addition, 3 dogs had heart murmurs and 1 had a third-degree atrioventricular block. Four dogs had myocarditis and 2 had pericarditis or epicarditis. Two dogs had cardiac signs attributed to extracardiac compression by fungal granulomas and clinical signs were relieved by treatment. Half of the remaining 6 dogs were euthanized; 2 of these were not treated. Of the remaining 3 dogs, 1 dog died acutely while sleeping; the second died intraoperatively during an attempt to place an epicardial pacemaker; and the third had Blastomyces-induced endocarditis and died of heart failure. Blastomycosis should be considered in the differential diagnosis of dogs from endemic areas with inflammatory myocarditis, heart block, heart base or intracardiac mass lesions, syncope, or endocarditis.

Solitary Liver Mass Detected by Transesophageal Echocardiography

Transesophageal echocardiography (TEE) has become a mainstay of cardiac surgery in the diagnosis of intracardiac and vascular lesions adjacent to the esophagus. In this case, we detected a liver mass during intraoperative TEE examination in a patient undergoing elective cardiac valve surgery. Preoperatively, the patient had normal liver function tests and no symptoms of hepatocellular carcinoma. This mass was diagnosed as hepatocellular carcinoma after the surgery and treated with transarterial chemoembolization. In conclusion, this case report outlines another potential application of intraoperative TEE extending its role outside the realm of cardiac surgery. A liver mass was detected during intraoperative transesophageal echocardiography (TEE) examination in a patient undergoing cardiac valve surgery. This incidental finding permitted the early diagnosis of hepatocellular carcinoma, and demonstrates another potential utility of TEE.

Mitral annular calcification and brown tumor of the rib in a child with chronic renal failure

Mitral annular calcification and liquefaction necrosis of this lesion mimicking intracardiac tumor because of secondary hyperparathyroidism have been described in adult patients with chronic renal failure, but have not been reported in children. Chronic renal failure is one of the predisposing factors of this condition. We report the case of a 13-year-old patient with continuous ambulatory peritoneal dialysis with severe hyperparathyroidism who was found to have intracardiac and rib lesions considered to be brown tumors.

Cardiopulmonary Bypass in Surgery for Interrupted Aortic Arch

Interrupted aortic arch is a complicated congenital heart defect. Because of its anatomic features, the conventional cardiopulmonary bypass (CPB) procedure is not suitable for the surgery of this type of lesion. Thus, we conducted a retrospective study of CPB in surgery for the disease. Ten patients with interrupted aortic arch underwent surgery by one of three different CPB methods, including profound hypothermia with circulatory arrest in four cases, profound hypothermia with low flow rate in five cases, and normothermia in one case. In profound hypothermic CPB, both ascending aorta and main pulmonary artery were cannulated. Through these two cannulas, the flow was pumped to the upper and lower body separately to cool down the body temperature. After cooling, the main pulmonary artery cannula was removed and interrupted aortic arch was corrected either under low flow rate perfusion or under circulatory arrest. After this, the other intracardiac lesions were repaired under conventional CPB. At the end of CPB, one patient demonstrated third-degree atria-ventricular block and required reinstituting CPB and a second procedure to repair the ventricular septal defect (VSD). In the intensive care unit, one patient developed lung infection and dyspnea after extubation that required intubation and mechanical ventilation for another several days. Another patient required 3 days of peritoneal dialysis caused by low cardiac output, hyperkalemia, and oliguria. All patients survived. The mechanical ventilation times were from 8 hours to 8 days and stays in the intensive care unit were from 4 to 12 days. Profound hypothermic cardiopulmonary bypass either with low flow rate or with circulatory arrest is equally the preferable choice for the surgery of interrupted aortic arch.

Inadvertent interruption of descending thoracic aorta on cardiopulmonary bypass during repair of a ventricular septal defect and interruption of a patent ductus arteriosus: Its recognition, consequences, and prevention

PATENT DUCTUS ARTERIOSUS (PDA) is commonly closed through a left thoracotomy. The sizes of the PDA, pulmonary artery (PA), and aorta can be quite similar, and to identify the ductus, the surgeon can test occlude the vessel to be ligated.1 Accidental interruption of the ascending aorta, pulmonary artery (PA) or descending thoracic aorta (DTA) can lead to devastating results. Placement of the pulse oximeter on a lower extremity and the blood pressure cuff on the right arm allow the anesthesiologist to assess flow in the ascending and descending aorta. Occlusion of the PA is characterized by a decrease in the arterial saturation and a decrease followed by an increase in end-tidal carbon dioxide tension.1 These methods of identification of the vessel test occluded are dependent on pulsatile blood flow and intermittent positive-pressure ventilation. During simultaneous repair of intracardiac lesions and interruption of PDA through a midsternotomy,2 PDA is ligated either before3 or after4 initiation of cardiopulmonary bypass (CPB). PDA is identified by its anatomic location, by its relationship with the left pulmonary artery and the undersurface of the aorta4 and by identifying its opening from within the pulmonary artery.2 On CPB, collapse of the PA and rise in the mean arterial pressure after ligation are the indirect features that suggest that the vessel occluded is the PDA. The monitoring used during PDA ligation through a left thoracotomy to prevent accidental ligation of a vessel other than the PDA is less useful during CPB because of the loss of pulsatile blood flow and discontinuation of ventilation. A case of fatal accidental ligation of DTA in a child who underwent ventricular septal defect (VSD) repair and PDA interruption under CPB is described.

Working heart off-pump cardiac repair (OPCARE) ? the next step in robotic surgery?

The objective of the present study was to assess the feasibility of working heart off-pump cardiac repair (OPCARE), for example, for simple congenital defects or valves, with the help of a commercially available surgical robot in order to identify problem areas and to explore potential solutions. OPCARE was studied in four bovine experiments using the Zeus (Computermotion, Santa Barbara, CA, USA) surgical robot providing 3D viewing of the extra cardiac instruments in combination with two intra-cardiac ultrasound visualisation systems (Clearview Ultra, Boston Scientific, La Garenne, France) and Accunav (Accuson, Mountain View, CA, USA) allowing for two different viewing planes of the heart and identification of both, intra-cardiac structures and intra-cardiac robotic instruments. For the given experimental set-up, introduction of robotic instruments into the right atrium (typical for instrumentation during OPCARE), grabbing of the second instrument (typical for removal of intra-cardiac foreign bodies), and grabbing of relatively still atrial structures, which can be visualised by intra-cardiac ultrasound (typical for resection of specific cardiac tissue, clipping of a specific cardiac structure, steered access to other cardiac cavities) have been realised with relative ease. In contrast, grabbing of moving cardiac structures like a-v valve leaflets (typical for valve testing and absolutely necessary for valve repair) required, in general, numerous attempts, but was finally successful in the majority of test animals. Finally, introduction of self-attaching suture material (U-clips, Coalescent Surgical, Sunnyvale, CA, USA) typical for edge-to-edge valve repair could not be performed in reproducible fashion with the equipment used. We conclude that OPCARE is feasible for simple intra-cardiac lesions using current robotic instrumentation and state of the art intra-cardiac ultrasound. However, more complex procedures require further development of both robotic instrumentation and intra-cardiac visualisation.

Volume quantification of intracardiac mass lesions by transesophageal three-dimensional echocardiography

As compared with two-dimensional (2-D) transesophageal echocardiography (TEE), 3-D echocardiography now permits more realistic visualization of cardiac anatomy and of intracardiac lesions. The aim of this study was to apply newer 3-D echocardiographic techniques to quantify volumes of intracardiac masses undergoing surgical resection seen during an intraoperative TEE. The calculated volumes were compared with actual in vitro measurements of surgically resected masses. A total of 14 patients (9 men; 5 women; age range between 21 and 77 years) with intracardiac mass lesions (4 tumors: 3 left atrial myxomas and 1 mitral valve fibroelastoma, and 10 vegetations: 5 aortic valve, 3 mitral valve, 1 tricuspid and 1 pulmonary valve) were studied. Using commercially available 3-D reconstruction software (TomTec v. 4.1), the volumes of intracardiac masses were estimated using both the average rotation (rotation around the long axis, AR) and disk summation (parallel short axis cuts, DS) methods. Volumes of these lesions were also measured in vitro by water submersion. They ranged from 0.20 mL to 24 mL (mean ± SD = 8.07 ± 9.21 mL). Both 3-D TEE AR and 3-D TEE DS calculated volumes correlated excellently with in vitro measured volumes (r = 1.00 and r = 0.98, respectively, p = < 0.0001). The correlation between 3-D TEE AR and 3-D TEE DS calculated volumes was also excellent (r = 0.98, p = < 0.0001). In conclusion, the volume assessments by 3-D TEE of intracardiac mass lesions correlated well with in vitro measured volumes of surgical specimens. This technique may prove to be valuable in further defining intracardiac pathology and is a further advancement toward the application of clinically useful 3-D echocardiography. (E-mail: nanda@uab.edu)

Assessment of the vascularity of a left ventricular mass using myocardial contrast echocardiography

In addition to a thrombus, an intracardiac mass lesion can represent a primary cardiac tumor, or a metastatic lesion to the heart. The echocardiographic appearance and location of the mass, as well as the associated clinical scenario provide useful clues regarding cause, but these echocardiographic features alone may not always be diagnostic. Because most benign cardiac tumors are relatively avascular, the vascularity of a mass may be a feature that can help differentiate benign from malignant tumors.We present a case of a patient with a history of malignant melanoma who was found incidentally to have a cardiac mass on echocardiography. Contrast echocardiography (CE) was used to evaluate the vascularity of the mass. This case is compared with another patient with an apical thrombus, which represents an “avascular” intracardiac structure.J Am Soc Echocardiogr 2002;15:1419-22.

Multiple intracardiac lesions after blunt chest trauma

Closed chest trauma can cause rupture of intracardiac structures. We report the case of a 17-year-old boy whose chest was trodden by a horse. He sustained rupture of tricuspid and aortic valve leaflets and rupture of the interventricular septum. He underwent surgical repair of these lesions, but aortic insufficiency developed 2 years later and the aortic valve was replaced with a mechanical prosthesis.

Cardiac Rhabdomyoma in an Adult Patient Presenting With Ventricular Arrhythmia

Cardiac rhabdomyomas are extremely uncommon in the adult patient. We describe a previously healthy man who presented with ventricular arrhythmias resulting from a right ventricular, cardiac rhabdomyoma. Echocardiography, CT scanning, and MRI are recognized as useful diagnostic modalities for intracardiac lesions. Cardiac catheterization in our patient demonstrated the presence of a tumor blush. This has not previously been reported with cardiac rhabdomyomas. Although lesions may spontaneously regress, surgery is often necessary and frequently resolves the underlying arrhythmia.

Cardiac Metastasis of Cloacagenic Carcinoma of the Vagina: A Case and Review of Gynecologic Malignancies with Cardiac Metastasis

We report of a case of a 44-year-old woman with a vaginal cloacagenic carcinoma who initially presented with a hymenal lesion that metastasized first to the perihilar lymph nodes and then consequently to the right ventricle. The embryological tumor is rare with only a few cases of vaginal or vulvar involvement. We present the first case of cloacagenic cancer of the vagina with metastasis to the heart. The lesion was surgically resected after completion of neoadjuvant therapy. Herein we present this unique case and the clinical manifestations of intracardiac and pericardial lesions from gynecologic malignancies.

Primary Reconstruction of Interrupted Aortic Arch - Surgical Management and Results

Type B interrupted aortic arch associated with other extracardiac and intracardiac congenital lesions is a lethal defect. Surgical management with a one- or two-stage approach is complex, difficult and associated with high morbidity and mortality. Between January 1996 and December 1999, 5 consecutive patients with type B interrupted aortic arch were operated on. The group included 3 girls and 2 boys weighing from 2.4 kg to 3.5 kg (mean 2.84 kg) and aged from 4 to 35 days (mean 15 days). All patients underwent a primary reconstruction of the aortic arch and one-stage repair of the associated heart defects. Two patients developed symptoms of mild aortic arch narrowing 7-12 months postoperatively. Balloon angioplasty of the obstructed site was successfully carried out in one of the patients. In the second child, angioplasty was not successful and surgery was used. All the patients survived and are being followed-up. This experience supports a strategy of primary one-stage repair of IAA type B, including direct anastomosis for aortic arch reconstruction, and all associated heart anomalies in the neonatal period.

Isolated fracture of the ventricular septum after blunt chest trauma

Isolated rupture of of the ventricular septum after blunt chest trauma is a very rare traumatic affection. A 21-year-old man was admitted to our hospital because of blunt chest trauma and a forearm fracture. Initial echocardiography did not show any intracardiac or extracardiac pathologic lesions, but 12 hours later this examination was repeated because of the onset of a holosystolic murmur. An unusual traumatic rupture of the ventricular septum was demonstrated. The hemodynamically stable condition of the patient allowed surgical repair to be performed 3 months later.

Minimally invasive cardiac surgery for intracardiac congenital lesions.

Minimally invasive cardiac surgery has recently been applied to the correction of intracardiac lesions. This report reviews our experience of minimally invasive cardiac surgery in 119 patients with intracardiac congenital lesions. From October 1995 to April 1997, 119 patients (48 male and 71 female, aged 0.9-65 years old, 18.5+/-17.8) received elective minimally invasive cardiac surgery at Chang Gung Memorial Hospital, Taipei, Taiwan for repair of atrial septal defect (96 patients) or ventricular septal defect (23 patients). The operations were performed through right submammary incision (ASD) or left parasternal minithoracotomy (VSD), under femoro-femoral or femoro-atrial cardiopulmonary bypass with fibrillatory arrest. All of the defects were repaired successfully. The bypass time was 25-125 min (46+/-18). The operation time was 1.5-5.2 h (2.8+/-0.8). The postoperative course was uneventful in all patients. Follow-up (1.0-18.2 months, mean 7.3) was complete, with no late deaths or residual shunt. All patients were found to be in NYHA functional class I or II. Our experience demonstrate that minimally invasive cardiac surgery is a technically feasible, safe, and effective procedure in surgical correction of selective simple intracardiac congenital lesions, yielding good short-term results.

Video-assisted minimal access in excision of left atrial myxoma

Background. Minimal access surgery with video-assisted endoscopy has been applied to the correction of intracardiac lesions. We report our experience using this technique in surgical excision of left atrial myxoma in 3 patients. Methods. From November 1995 to March 1997, 3 female patients, ages 45 to 80 years (mean, 62.7 years), received emergency operations for excision of left atrial myxoma. These operations were performed through a right anterior submammary minithoracotomy or right parasternal incision with the assistance of endoscopy during femoro–femoral cardiopulmonary bypass. The myocardium was protected by continuous coronary perfusion with fibrillatory arrest or cardioplegic arrest with aortic cross-clamping. Results. All the tumors were excised completely through the right atrial approach. The bypass time was 92 to 148 minutes (mean, 111 minutes). The operation time was 3.2 to 4.4 hours (mean, 3.7 hours). There were no hospital deaths. Follow-up, which ranged from 6 to 19 months (mean, 10.5 months), was complete in all patients. Transthoracic echocardiographic examination showed good ventricular function without any residual tumors. Patients were found to be in New York Heart Association functional class I or II. They were satisfied with the good cosmetic healing of the incision. Conclusions. Our experience demonstrates that minimal access surgery is a technically feasible, safe, and effective procedure in surgical excision of left atrial myxoma.