The Texas Heart Institute community is saddened by the loss of John C. Norman, MD, who died at age 84 on 23 August 2014. He was the first director of the THI Cullen Cardiovascular Surgical Research Laboratories and was the founder and first editor-in-chief of this journal. John Clavon Norman, Jr., was born on 11 May 1930, in Charleston, West Virginia. His father was an architect and structural engineer, and his mother was a high school English teacher. As one of West Virginia's first licensed black architects, John C. Norman, Sr., erected hotels, high schools, theaters, hospitals, and churches around
As a full-time medical educator for more than half a century, I have learned that good doctors have certain defining qualities—a love for the medical profession, a passionate quest for knowledge, the constant pursuit of excellence, high regard for honesty, hard work, humility, and putting patients first, always. In spite of their obvious values, these qualities receive only lip service—if that—during medical school and postgraduate training. Moreover, another crucial quality of good doctors—skepticism—gets even less attention. In fact, medical schools and postgraduate training programs actually stifle skeptical attitudes among trainees.1 I find, for example, that medical students, house officers,
As an MD/PhD student, I have always been fascinated by the basic science aspects of medicine. Throughout my preclinical years and PhD training, my focus was largely on the technical details of the inner workings of the body. I thought that understanding the structure and pathophysiology of the human body, and knowing how to fix it when it was
Transradial access is an alternative to the transfemoral approach in coronary interventions. It results in less access-site bleeding, shorter hospital stays, lower costs, and less pain for the patient. However, some authors have suggested that the transradial approach might lead to radial artery occlusion, which precludes repeated same-artery catheterizations. Using data from our center, we evaluated the feasibility, safety, and efficacy of repeated transradial catheterization. We reviewed the 3,006 transradial catheterizations performed at our center from 2006 through 2009. Patients who had undergone at least one repeated transradial catheterization were identified, their cases monitored through 2012, and their baseline characteristics and other factors, including procedural sequelae, were analyzed. Seventy-nine patients underwent repeated right radial artery catheterizations, for a total of 92 repeated procedures. Repeated access to the right radial artery was not achieved in 4 attempts (failure rate, 4.3%), because of poor pulses or the operator's inability to advance the wire. No major sequelae were noted. The average times between the 1st to 2nd, 2nd to 3rd, and 3rd to 4th catheterizations were 406, 595, and 401 days, respectively. Our procedural success rate of 95.7% in performing repeated transradial catheterizations with no major sequelae provides support for the efficacy and safety of such procedures.
Major medical society guidelines recommend the measurement of fractional flow reserve (FFR) as an aid in choosing percutaneous coronary intervention in patients with stable coronary artery disease. We investigated the measurement of FFR among interventionalists, analyzing operators' attributes and decision-making processes to reveal differences in their applications of FFR and the reasons for those differences. An electronic survey study of 1,089 interventionalists was performed from 2 February through 6 March 2012, yielding 255 responses. Most respondents were >45 years old (58%), worked primarily in a community hospital (59%), and performed 10 to 30 cases per month (52%). More than half (145/253, 57%) used FFR measurement in less than one third of cases, and 39 of 253 (15%) never used it. There were no differences in use of FFR by age, practice location, or angiogram volume (P >0.05 for all). Respondents used FFR measurement more frequently than intravascular ultrasonography (73% vs 60%) to help guide the decision to stent (P <0.01). Operators reported that their primary reasons for not using FFR were lack of availability (47%) and problems with reimbursement (39%). There was no difference in FFR use by operator age, practice setting, or case volume.
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.
The aim of this study was to evaluate the embolic sequelae of left atrial myxomas and their influence on diagnosis, treatment, and prognosis. Seventy-eight patients were retrospectively investigated. According to their symptoms and neurologic-imaging findings, these patients were classified into 2 groups: embolism (15 patients, 19%) and nonembolism (63 patients, 81%). The time from the first onset of symptoms to diagnosis (that is, the duration of symptoms) was significantly longer in the embolism group than in the nonembolism group (105 ± 190 vs 23 ± 18 d; P <0.01). The myxomas were divided into 2 types on the basis of clinicopathologic findings: type 1, with an irregular or villous surface and a soft consistency, and type 2, with a smooth surface and a compact consistency. There were 42 patients with type 1 myxoma and 36 with type 2. Type 1 myxoma was more frequently found in the embolism group (12 patients, 29%) than was type 2 myxoma (3 patients, 8%). The difference was significant (P=0.04). There were 2 perioperative deaths in the nonembolism group. No recurrence of cardiac myxoma or death was recorded in either group during follow-up. In the embolism group, neurologic symptoms were relieved by surgery, and no subsequent neurologic event was reported. Because surgical resection is highly effective in left atrial myxoma, we should strive for early diagnosis in order to shorten the duration of symptoms and to avoid worse neurologic damage in patients in whom an embolic event is the initial manifestation.
The brachiocephalic artery is an alternative cannulation site in the repair of ascending aortic lesions that require circulatory arrest. We evaluate the effectiveness and safety of this technique. Proximal aortic surgery was performed in 32 patients from 2006 through 2012 via brachiocephalic artery cannulation and circulatory arrest. Twenty-four (75%) of the patients were men. The mean age was 48.69 ± 9.43 years (range, 30–68 yr). Twelve had type I dissection, 2 had type II dissection, and 18 had true aneurysms of the ascending aorta. All operations were performed through a median sternotomy. The arterial cannula was inserted through an 8-mm vascular graft anastomosed to the brachiocephalic artery in an end-to-side fashion. In dissections, the distal anastomosis was performed without clamping the aorta. The patients were cooled to 24 °C, and circulatory arrest was established. The brachiocephalic and left carotid arteries were clamped, and antegrade cerebral perfusion was started at a rate of 10 mL/kg/min. Cardiopulmonary bypass was resumed after completion of the distal anastomosis and the initiation of rewarming. The proximal anastomosis was then performed. None of the patients sustained a major neurologic deficit, but 5 patients experienced transient postoperative agitation (<24 hr). There were 2 early deaths (6.25%), on the 3rd and the 11th postoperative days, both unrelated to the cannulation technique. Brachiocephalic artery cannulation through a graft can be a safe and effective technique in proximal aortic surgical procedures that require circulatory arrest.
We report our identification of a single coronary ostium arising from the right coronary sinus of Valsalva, in a 63-year-old woman who presented with chest pain atypical of angina. Coronary angiograms showed that the left anterior descending coronary artery arose from a right ventricular branch and that the left circumflex coronary artery arose from a right posterolateral branch. Both arteries reconstituted themselves in a backward fashion from the apex to the base of the heart—a configuration that to our knowledge has not been reported. The patient was treated conservatively and reported no chest pain 24 months later.
Giant coronary artery aneurysms are rare, with a reported prevalence of 0.02% to 0.2%. Causative factors include atherosclerosis, Takayasu arteritis, congenital disorders, Kawasaki disease, and percutaneous coronary intervention. Most giant coronary artery aneurysms are asymptomatic, but some patients present with angina pectoris, sudden death, fistula formation, pericardial tamponade, compression of surrounding structures, or congestive heart failure. Clinical sequelae include thrombus formation, embolization, fistula formation, and rupture. Surgical correction is generally accepted as the preferred treatment for giant coronary artery aneurysms. We present an illustrative case of a giant 70 × 40-mm coronary artery aneurysm in a 56-year-old man who declined surgery and died one month later. In addition, we provide a review of the medical literature on giant coronary artery aneurysms.
The MitraClip percutaneous mitral valve repair system, developed as an option for percutaneous mitral repair, was clinically introduced in 2007. From 2010 through 2012, 6 of our patients underwent mitral valve surgery after MitraClip failure. Their mean age was 75 ± 7.7 years (range, 62–87 yr). Three had undergone cardiac surgery previously. In 5 of the 6 patients, mitral regurgitation recurred after initially successful MitraClip deployment and was the indication for surgery. The mean interval between MitraClip implantation and surgery was 106 ± 86 days (range, 0–238 d). Mitral valve repair was feasible in 3 patients; the others underwent valve replacement. All the patients underwent additional cardiac procedures, because the MitraClip worsened existing conditions. Echocardiograms revealed sufficient valvular repairs. Two patients died during hospitalization, one of cerebral infarction and the other of bowel ischemia. Mitral valve repair after failed MitraClip therapy can be complex and a surgical challenge. Careful consideration should be given to appropriate patient selection for MitraClip therapy, because the MitraClip can cause existing pathologic valvular conditions to deteriorate substantially. The interval between MitraClip failure and corrective surgery should be as short as possible. The primary indication is an issue of ongoing discussion.
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.
Cardiac tumors can lead to distinct electrocardiographic changes and ventricular arrhythmias. Benign and malignant cardiac tumors have been associated with ventricular tachycardia. When possible, benign tumors should be resected when ventricular arrhythmias are intractable. Chemotherapy can shrink malignant tumors and eliminate arrhythmias. We report the case of a 52-year-old woman with breast sarcoma whom we diagnosed with myocardial metastasis after she presented with palpitations. The initial electrocardiogram revealed sinus rhythm with new right bundle branch block and ST-segment elevation in the anterior precordial leads. During telemetry, hemodynamically stable, sustained ventricular tachycardia with right ventricular localization was detected. Images showed a myocardial mass in the right ventricular free wall. Amiodarone suppressed the arrhythmia. To our knowledge, this is the first report of ventricular tachycardia associated with radiation-induced undifferentiated sarcoma. We discuss the distinct electrocardiographic changes and ventricular arrhythmias that can be associated with cardiac tumors, and we review the relevant medical literature.
Lymphocytic neoplasm involving the heart is not common and usually presents with pericardial effusion or focal myocardial infiltration. Myocardial infarctions due to leukemic infiltration of the coronary arteries are rarely reported. We present the case of a 52-year-old Guatemalan man with a one-year history of untreated T-cell prolymphocytic leukemia. He was admitted to our hospital for chemotherapy and evaluation of a pulmonary cavitary lesion by wedge resection. During sedation, the patient experienced acute respiratory failure and hypovolemic shock, from which he could not be resuscitated. Autopsy revealed that leukemic cells extensively infiltrated the aorta, myocardium, and coronary arteries. The lumina of the 3 major coronary artery branches showed 70% to 95% stenosis, with multifocal remote myocardial infarctions. Tumor cells were also detected in the lungs and other organs. The acute cardiorespiratory insufficiency secondary to leukemia—particularly the extensive infiltration of the coronary arteries and myocardium, and the multiple myocardial infarctions—eventually resulted in cardiac death.
Vasovagal (neurocardiogenic) syncope, a subtype of reflex syncope, has many well-known triggers. However, we found no previous report of vasovagal exercise-induced syncope in a sedentary person. We present the case of a 35-year-old sedentary woman who experienced vasovagal syncope as she underwent an exercise stress test. Results of evaluations, including resting and stress electrocardiography and echocardiography, were normal. Her presentation is highly unusual: syncope has typically not been associated with exercise except in young athletes, people with structural heart abnormalities, or people with a prolonged QT syndrome. To our knowledge, this is the first report of vasovagal syncope associated with exercise in a sedentary patient who had normal cardiac and electrophysiologic function. We suggest possible physiologic mechanisms and diagnostic strategies.
The rupture of a mycotic femoral artery pseudoaneurysm in an intravenous drug abuser is a limb- and life-threatening condition that necessitates emergency intervention. Emergency stent-grafting appears to be a viable, minimally invasive alternative, or a bridge, to subsequent open surgery. Caution is required in cases of suspected concomitant deep vein thrombosis in order to minimize the possibility of massive pulmonary embolism during stent-grafting, perhaps by omitting stent-graft postdilation or by inserting an inferior vena cava filter first. We describe the emergency endovascular management, in a 60-year-old male intravenous drug abuser, of a ruptured mycotic femoral artery pseudoaneurysm, which was complicated by a fatal pulmonary embolism.
Although chest pain in association with ST-segment electrocardiographic deviation is often indicative of cardiac ischemia, it has also been associated with noncardiac conditions. The case of a 63-year-old woman that we report here is extraordinary because her presentation of “acute abdomen” did not appear severe enough to warrant urgent surgical intervention, whereas the chest pain and electrocardiographic changes (supported by rising troponin levels) created strong clinical suspicion of acute coronary syndrome. Was the evidence of cardiac ischemia a primary event, or was it a sequela of an acute surgical condition? Noncardiac surgical cases associated with evidence of myocardial injury can be extremely challenging from a diagnostic and management perspective. We believe that the accuracy of the clinical diagnosis is crucial to a well-considered approach.
We report the case of an 85-year-old woman with severe aortic stenosis who underwent transcatheter aortic valve replacement with use of the Edwards Sapien® valve system. The procedure was complicated by rupture of the valve-deployment balloon, with separation and retention of the nose cone of the RetroFlex 3® delivery system in the iliac artery. Our endovascular retrieval of the equipment was successful, and we achieved access-site hemostasis by deploying a covered stent. To our knowledge, this is the first report of the endovascular retrieval of a malfunctioning delivery system during transcatheter aortic valve replacement.
Heparin-induced thrombocytopenia is a well-recognized complication of anticoagulation with heparin. We present the case of a patient with recent heparin-induced thrombocytopenia who subsequently needed surgery on an emergency basis for acute type A aortic dissection. This article reports the successful use of bivalirudin, a direct thrombin inhibitor, as an alternative to heparin throughout cardiopulmonary bypass and deep hypothermic circulatory arrest. We contend that bivalirudin is a safe alternative to heparin when performing surgery for aortic dissection and should be considered as an option for use in patients who present with heparin-induced thrombocytopenia.
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.
The left internal mammary artery is the preferred graft for treating left anterior descending coronary artery disease. Dissection is a rare but grave sequela of internal mammary artery graft angiography. The available medical literature is scant, perhaps as a result of under-reporting. We report a case in which dissection of the internal mammary artery graft occurred during diagnostic angiography, and we discuss its management. In addition, we review the available literature and provide a retrospective analysis of the data from our own catheterization laboratory. In our single-center analysis of 542 cases of selective internal mammary artery graft angiography, we found only the single case of internal mammary artery graft dissection (0.2%) that we report here. Our review of the literature revealed 7 reported cases of internal mammary artery graft dissection, 3 of which were iatrogenic. There were no identifiable risk factors for such dissection. After treatment with angioplasty and stenting, all patients had good outcomes during follow-up.
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.
Pheochromocytoma should be considered in young patients who have acute cardiac decompensation, even if they have no history of hypertension. Atrioventricular node ablation and pacemaker placement should be considered for stabilizing pheochromocytoma patients with cardiogenic shock due to atrial tachyarrhythmias. A 38-year-old black woman presented with cardiogenic shock (left ventricular ejection fraction, <0.15) that did not respond to the placement of an intra-aortic balloon pump. A TandemHeart® Percutaneous Ventricular Assist Device was inserted emergently. After atrioventricular node ablation and placement of a temporary pacemaker, the TandemHeart was removed. Computed tomography of the abdomen revealed a pheochromocytoma. After placement of a permanent pacemaker, the patient underwent a right adrenalectomy. This is, to our knowledge, the first reported case of pheochromocytoma-induced atrial tachyarrhythmia that led to cardiogenic shock and cardiac arrest unresolved by the placement of 2 different ventricular assist devices, but that was completely reversed by radiofrequency ablation of the atrioventricular node and the placement of a temporary pacemaker. We present the patient's clinical, laboratory, and imaging findings, and we review the relevant literature.
Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.
Infective endocarditis complicated by abscess formation and coronary artery compression is a rare clinical event with a high mortality rate, and diagnosis requires a heightened degree of suspicion. We present the clinical, angiographic, and echocardiographic features of a 73-year-old woman who presented with dyspnea and was found to have right coronary artery compression that was secondary to abscess formation resulting from diffuse infectious endocarditis. We discuss the patient's case and briefly review the relevant medical literature. To our knowledge, this is the first reported case of abscess formation involving a native aortic valve and the right coronary artery.
A 69-year-old woman presented with hematuria. Magnetic resonance imaging revealed a left renal mass extending into the retrohepatic inferior vena cava (IVC) (Figs. 1 and 2). Both magnetic resonance imaging of the brain and a bone scan were negative for distant metastases. Intraoperatively, the IVC was cross-clamped below the level of the hepatic veins to prevent dissemination of the intracaval part of the tumor. Left radical nephrectomy and resection of the intracaval part of the tumor mass were successfully performed. The IVC was repaired primarily. The patient tolerated the procedure well and was discharged from the
A 28-year-old woman presented with a history of uncontrolled hypertension, despite medical therapy, and with fibromuscular dysplasia of the renal arteries (RAs). Angiograms from another hospital revealed an unusual left RA arising vertically from the aorta. The left RA had a distinctive beaded appearance in its proximal and mid segments, consistent with fibromuscular dysplasia. At that time, the patient underwent successful balloon angioplasty. Because of her persistently elevated blood pressure, we performed repeat renal and abdominal angiography. The ostial, proximal, and mid portions of the left RA were patent. In addition, we identified substantial anatomic variations of the abdominal aorta
A 15-year-old boy with a history of Kawasaki disease presented after an exercise nuclear stress test (performed before a contemplated weight-loss program) revealed a reversible inferolateral and apical defect (left ventricular ejection fraction, 0.53). A coronary angiogram showed the right coronary artery with an 11.5 × 6 × 5-mm proximal aneurysm, proximal 50% stenosis, and mid 70% stenosis; the left anterior descending coronary artery (LAD) with a proximal 60% stenosis followed by an aneurysm; and the left circumflex coronary artery with a small aneurysm of the proximal segment (Fig. 1). We placed drug-eluting stents in the proximal and
To the Editor: In the operating room, the ambient temperature might be uncomfortably warm for the surgical team, for several reasons. During off-pump surgery, the temperature might purposely be kept high. Without use of the heart-lung machine, it is important to avoid overcooling the patient, because rewarming can be difficult and take time. In tropical countries during summer, the atmospheric temperature might be so high that effective indoor cooling cannot be achieved. In some countries, power outages occur frequently, and uninterrupted electrical power is allocated to crucial infrastructure. Meanwhile, the surgical team is gowned and needs to stay focused. We
To the Editor: We appreciated the article by Katukuri and colleagues1 about the 37-year-old man who had left ventricular hypertrabeculation/noncompaction (LVHT) associated with polycystic kidney disease (PCKD). We wish to present some considerations that the authors might have been unable to discuss, given the length criteria for Images in Cardiovascular Medicine publications in the Journal. The authors speculate that the PCKD was caused by mutations in the patient's PCKD1 or PCKD2 genes. Polycystic kidney disease can arise from causes other than PCKD mutations.2 Renal cysts might be a
