Daniel Murphy

All Publications

• Risk Estimates for Atherosclerotic Cardiovascular Disease in Adults With Congenital Heart Disease AMERICAN JOURNAL OF CARDIOLOGY Lui, G. K., Rogers, I. S., Ding, V. Y., Hedlin, H. K., MacMillen, K., Maron, D. J., Sillman, C., Romfh, A., Dade, T. C., Haeffele, C., Grady, S. R., McElhinney, D. B., Murphy, D. J., Fernandes, S. M. 2017; 119 (1): 112-118

  Abstract

  The adult with congenital heart disease (CHD) is at risk of developing atherosclerotic cardiovascular disease (ASCVD). We performed a cross-sectional study to describe established ASCVD risk factors and estimate 10-year and lifetime risk of ASCVD in adults over age 18 with CHD of moderate or great complexity using 3 validated risk assessment tools-the Framingham Study Cardiovascular Disease Risk Assessment, the Reynolds Risk Score, and the ASCVD Risk Estimator. We obtained extensive clinical and survey data on 178 enrolled patients, with average age 37.1 ± 12.6 years, 51% men. At least 1 modifiable ASCVD risk factor was present in 70%; the 2 most common were overweight/obesity (53%) and systemic hypertension (24%). Laboratory data were available in 103 of the 178 patients. Abnormal levels of glycated hemoglobin, high-sensitivity C-reactive protein, and high-density lipoprotein were each found in around 30% of patients. The 10-year ASCVD predicted risk using all 3 tools was relatively low (i.e., at least 90% of patients <10% risk), yet the median estimated lifetime risk was 36%. In conclusion, ASCVD risk factors are prevalent in adults with CHD. The risk estimation tools suggest that this population is particularly vulnerable to ASCVD with aging and should undergo guideline-based screening and management of modifiable risk factors.

  View details for DOI 10.1016/j.amjcard.2016.09.023

  View details for Web of Science ID 000391246900018

  View details for PubMedID 28247847

  View details for PubMedCentralID PMC5334785

• Surgical Repair of 115 Patients With Anomalous Aortic Origin of a Coronary Artery From a Single Institution. World journal for pediatric & congenital heart surgery Mainwaring, R. D., Murphy, D. J., Rogers, I. S., Chan, F. P., Petrossian, E., Palmon, M., Hanley, F. L. 2016; 7 (3): 353-359

  Abstract

  Anomalous aortic origin of a coronary artery (AAOCA) has been associated with myocardial ischemia and sudden death. The past decade has provided important insights into the natural history and typical patterns of presentation. However, there are also a number of unresolved controversies regarding the indications for surgery and the efficacy of that surgery. The purpose of this study was to review our surgical experience with AAOCA in 115 patients at a single institution.One hundred and fifteen patients have undergone surgical repair of AAOCA at our institution. There were 82 males and 33 females, and the median age at surgery was 16 years. Fifty-nine patients had preoperative symptoms of myocardial ischemia, including 56 with exertional chest pain or syncope and 3 sudden death events. Twenty-four patients had associated congenital heart defects. Seven patients had an associated myocardial bridge.Surgical repair was accomplished by unroofing of an intramural coronary in 86, reimplantation in 9, and pulmonary artery translocation in 20. There has been no early or late mortality. Fifty-seven (97%) of the 59 symptomatic patients have been free of any cardiac symptoms postoperatively. Two patients had recurrent symptoms and underwent reoperation (one had revision of the initial repair and one had repair of a myocardial bridge).Surgical repair of AAOCA can be safely performed and is highly efficacious in relieving symptoms of myocardial ischemia. The two "surgical failures" in this series had an anatomic basis and underscore the need to reassess both the proximal and distal anatomy in these patients.

  View details for DOI 10.1177/2150135116641892

  View details for PubMedID 27142404

• A recurrent fibrillin-1 mutation in severe early onset Marfan syndrome. Journal of pediatric genetics Sureka, D., Stheneur, C., Odent, S., Arno, G., Murphy, D., Bernstein, J. A. 2014; 3 (3): 157-162

  Abstract

  The recurrent substitution of isoleucine for threonine at codon 1048 (I1048T) substitution has been linked to severe, early onset Marfan syndrome, however, the existence of strong genotype-phenotype associations in Marfan syndrome (MFS) is not widely agreed upon. Our aim is to substantiate the association between the I1048T substitution and a severe clinical presentation to facilitate care planning and genetic counseling. We review the clinical findings from seven cases of early-onset MFS with a recurrent I1048T substitution. The presented findings include those from one newly diagnosed case, significant new detail from three additional cases, and a review of published findings in three cases. All seven individuals with the I1048T substitution had mitral insufficiency, arachnodactyly and characteristic facies consistent with early-onset MFS. Our findings support the existence of a genotype-phenotype correlation between the I1048T substitution and early-onset MFS. Recognition of this relationship has implications for genetic counseling and clinical care. Additionally, exploration of how the I1048T substitution results in a severe phenotype may lead to further insight into the pathophysiology of MFS.

  View details for DOI 10.3233/PGE-14095

  View details for PubMedID 27625872

• Heterotaxy syndromes and abnormal bowel rotation. Pediatric radiology Newman, B., Koppolu, R., Murphy, D., Sylvester, K. 2014; 44 (5): 542-551

  Abstract

  Bowel rotation abnormalities in heterotaxy are common. As more children survive cardiac surgery, the management of gastrointestinal abnormalities has become controversial.To evaluate imaging of malrotation in heterotaxy with surgical correlation and provide an algorithm for management.Imaging reports of heterotaxic children with upper gastrointestinal (UGI) and/or small bowel follow-through (SBFT) were reviewed. Subsequently, fluoroscopic images were re-reviewed in conjunction with CT/MR studies. The original reports and re-reviewed images were compared and correlated with surgical findings.Nineteen of 34 children with heterotaxy underwent UGI, 13/19 also had SBFT. In 15/19 reports, bowel rotation was called abnormal: 11 malrotation, 4 non-rotation, no cases of volvulus. Re-review, including CT (10/19) and MR (2/19), designated 17/19 (90%) as abnormal, 10 malrotation (abnormal bowel arrangement, narrow or uncertain length of mesentery) and 7 non-rotation (small bowel and colon on opposite sides plus low cecum with probable broad mesentery). The most useful CT/MR findings were absence of retroperitoneal duodenum in most abnormal cases and location of bowel, especially cecum. Abnormal orientation of mesenteric vessels suggested malrotation but was not universal. Nine children had elective bowel surgery; non-rotation was found in 4/9 and malrotation was found in 5/9, with discrepancies (non-rotation at surgery, malrotation on imaging) with 4 original interpretations and 1 re-review.We recommend routine, early UGI and SBFT studies once other, urgent clinical concerns have been stabilized, with elective laparoscopic surgery in abnormal or equivocal cases. Cross-sectional imaging, usually obtained for other reasons, can contribute diagnostically. Attempting to assess mesenteric width is important in differentiating non-rotation from malrotation and more accurately identifies appropriate surgical candidates.

  View details for DOI 10.1007/s00247-013-2861-4

  View details for PubMedID 24419494

• Unexplained double-chambered left ventricle associated with contracting right ventricular aneurysm and right atrial enlargement. Echocardiography (Mount Kisco, N.Y.) Finocchiaro, G., Murphy, D., Pavlovic, A., Haddad, F., Shiran, H., Sinagra, G., Ashley, E. A., Knowles, J. W. 2014; 31 (3): E80-4

  Abstract

  In this article, we describe a double-chambered left ventricle (LV) associated with a functional right ventricular (RV) aneurysm and right atrial (RA) enlargement in an asymptomatic 24-year-old woman with a family history of sudden cardiac death. We will discuss the differential diagnosis, genetic testing and possible prognostic implications.

  View details for DOI 10.1111/echo.12467

  View details for PubMedID 24299065

• Unexplained double-chambered left ventricle associated with contracting right ventricular aneurysm and right atrial enlargement. Echocardiography (Mount Kisco, N.Y.) Finocchiaro, G., Murphy, D., Pavlovic, A., Haddad, F., Shiran, H., Sinagra, G., Ashley, E. A., Knowles, J. W. 2014; 31 (3): E80-4

  View details for DOI 10.1111/echo.12467

  View details for PubMedID 24299065

• Coarctation of the Aorta Clinical Management of Congenital Heart Disease from Infancy to Adulthood Murphy, D. J. Cardiotext Publishing. 2014; 1st: 45–64
• Midterm Results of the Modified Ross/Konno Procedure in Neonates and Infants ANNALS OF THORACIC SURGERY Maeda, K., Rizal, R. E., Lavrsen, M., Malhotra, S. P., Akram, S. A., Davies, R., Suleman, S., Reinhartz, O., Murphy, D. J., Hanley, F. L., Reddy, V. M. 2012; 94 (1): 156-163

  Abstract

  The management of congenital aortic stenosis in neonates and infants continues to be a surgical challenge. We have performed the modified Ross-Konno procedure for patients who have severe aortic insufficiency or significant residual stenosis after balloon aortic dilation. The midterm results of this procedure were evaluated in this subset of patients.Between 1994 and 2010, a total of 24 patients younger than 1 year of age underwent the modified Ross-Konno procedure. The diagnoses were aortic stenosis with or without subaortic stenosis (n = 16), Shone's complex (n = 7), and interrupted aortic arch with subaortic stenosis (n = 1). The aortic root was replaced with a pulmonary autograft, and the left ventricular outflow tract (LVOT) was enlarged with a right ventricular infundibular free wall muscular extension harvested with the autograft.Age at operation ranged from 1 to 236 days (median 28 days). The median follow-up period was 81 months (range 1-173 months). There was 1 early death and no late mortality. Overall the 1-, 2-, and 5-year survival rate was 95% ± 4.5%. Freedom from aortic stenosis was 94.7% ± 5.1% at 1, 2, and 5 years. Less than mild aortic insufficiency was 93.3% ± 6.4% at 2 years, and 74.7% ± 12.9% at 5 years. In total, 23 reoperations and reinterventions were performed; 14 were allograft conduit replacements. Two patients required aortic valve plasty. None required valve replacement. The reintervention-free rate was 64.6% ± 10.8% at 2 years and 36.9% ± 11.3% at 5 years.Pulmonary autografts demonstrated good durability with low mortality and morbidity. This study shows that the modified Ross-Konno procedure can be a practical choice in selective cases for complex LVOT stenosis in neonates and infants.

  View details for DOI 10.1016/j.athoracsur.2012.03.007

  View details for Web of Science ID 000305801600033

  View details for PubMedID 22626750

• Screening for Aortic Root Dilation in Marfan Syndrome Using the Ratio of the Aortic Root to Descending Aortic Diameters in Children JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Kemna, M. S., Murphy, D. J., Silverman, N. H. 2009; 22 (10): 1109-1113

  Abstract

  The aim of this study was to develop a simpler echocardiographic screening tool for aortic root dilation in pediatric patients with Marfan syndrome. Aortic root dilation represents the most common cause of morbidity and mortality in patients with Marfan syndrome. It is traditionally defined by nomograms, relating aortic root dimensions to height or body surface area. In this study, the descending aorta (DAo) was used as an internal reference, thereby eliminating the need for nomograms when screening for aortic root dilation.The diameters of the aortic root at the sinus of Valsalva and the DAo were measured in 35 patients with Marfan syndrome and 52 normal controls in the parasternal long-axis echocardiographic view. The root/DAo ratio was calculated.A root/DAo ratio > or = 2 provided optimal sensitivity for root dilation (100%), whereas a root/DAo ratio > or = 2.3 provided optimal specificity (100%).The root/DAo ratio provides a simple, rapid, and valuable screening test for aortic root dilation, independent of nomograms and body size.

  View details for DOI 10.1016/j.echo.2009.06.002

  View details for Web of Science ID 000270367900003

  View details for PubMedID 19647408

• Surgically palliated double-inlet left ventricle with transposition of the great arteries mistaken for aortic aneurysm with dissection INTERNATIONAL JOURNAL OF CARDIOLOGY Gupta, A., Fleischmann, D., Murphy, D. J., Wu, J. C. 2008; 128 (2): E82-E84

  Abstract

  We report a 23-year-old male with history of double-inlet single ventricle with transposition of the great arteries who is s/p pulmonary artery banding, a Damus-Kaye-Stanzel anastomosis, and Fontan procedure during infancy and childhood who now presents with chest pain. A chest CTA at an outside hospital was thought concerning for the presence of a thoracic aortic aneurysm with dissection, prompting immediate transfer to our institution. However, repeat chest CTA at our institution revealed the predicted anastomoses based on his surgical procedures, which was misinterpreted as aortic aneurysm and dissection. An understanding of the physiology of his surgically repaired congenital heart disease is critical in interpreting his subsequent chest CTA and arriving at the appropriate clinical conclusion.

  View details for DOI 10.1016/j.ijcard.2007.04.165

  View details for Web of Science ID 000257950500043

  View details for PubMedID 17689761

• Does size matter? Clinical applications of scaling cardiac size and function for body size CIRCULATION Dewey, F. E., Rosenthal, D., Murphy, D. J., Froelicher, V. F., Ashley, E. A. 2008; 117 (17): 2279-2287

  Abstract

  Extensive evidence is available that cardiovascular structure and function, along with other biological properties that span the range of organism size and speciation, scale with body size. Although appreciation of such factors is commonplace in pediatrics, cardiovascular measurements in the adult population, with similarly wide variation in body size, are rarely corrected for body size. In this review, we describe the critical role of body size measurements in cardiovascular medicine. Using examples, we illustrate the confounding effects of body size. Current cardiovascular scaling practices are reviewed, as are limitations and alternative relationships between body and cardiovascular dimensions. The experimental evidence, theoretical basis, and clinical application of scaling of various functional parameters are presented. Appropriately scaled parameters aid diagnostic and therapeutic decision making in specific disease states such as hypertrophic cardiomyopathy and congestive heart failure. Large-scale studies in clinical populations are needed to define normative relationships for this purpose. Lack of appropriate consideration of body size in the evaluation of cardiovascular structure and function may adversely affect recognition and treatment of cardiovascular disease states in the adult patient.

  View details for DOI 10.1161/CIRCULATIONAHA.107.736785

  View details for Web of Science ID 000255394300014

  View details for PubMedID 18443249

• Right ventricular function in cardiovascular disease, Part II - Pathophysiology, clinical importance, and management of right ventricular failure CIRCULATION Haddad, F., Doyle, R., Murphy, D. J., Hunt, S. A. 2008; 117 (13): 1717-1731

  View details for DOI 10.1161/CIRCULATIONAHA.107.653584

  View details for Web of Science ID 000254576500013

  View details for PubMedID 18378625

• Right ventricular function in cardiovascular disease, part I - Anatomy, physiology, aging, and functional assessment of the right ventricle CIRCULATION Haddad, F., Hunt, S. A., Rosenthal, D. N., Murphy, D. J. 2008; 117 (11): 1436-1448

  View details for DOI 10.1161/CIRCULATIONAHA.107.653576

  View details for Web of Science ID 000254065200011

  View details for PubMedID 18347220

• Right coronary cameral fistula resulting from surgery of double chamber right ventricle. Journal of the American Society of Echocardiography Leeper, N. J., Gupta, A., Murphy, D. J., Wu, J. C. 2006; 19 (9): 1191 e9-11

  View details for PubMedID 16950481

• Transposition of the great arteries: long-term outcome and current management. Current cardiology reports Murphy, D. J. 2005; 7 (4): 299-304

  Abstract

  There is a large group of young adults who survived atrial baffle repair of transposition of the great arteries. Most survivors are asymptomatic, although nearly all have decreased exercise capacity. Loss of sinus rhythm and atrial arrhythmias are common and increase with age. There is concern about the ability of the right ventricle to function long term as a systemic pump, and recent publications have highlighted right ventricular dysfunction in this patient population. Sudden death and congestive heart failure are the main causes of death, and outcomes beyond 30 years are unknown. Pulmonary artery banding, late arterial switch, and cardiac transplantation are employed when intractable arrhythmias or right ventricular failure threaten survival or quality of life.

  View details for PubMedID 15987628

• Survey of specialized tertiary care facilities for adults with congenital heart disease INTERNATIONAL JOURNAL OF CARDIOLOGY Niwa, K., Perloff, J. K., Webb, G. D., Murphy, D., Liberthson, R., Warnes, C. A., Gatzoulis, M. A. 2004; 96 (2): 211-216

  Abstract

  Specialized tertiary care facilities developed in response to the increasing numbers of adults with congenital heart disease (CHD). Because this patient population comprises a relatively new area of specialized cardiovascular interest, the first facilities necessarily evolved without preexisting guidelines or interaction.To characterize the major features of the six original and largest tertiary adults CHD facilities.Written questionnaire sent to six participating facilities in North America and Europe. Information was analyzed centrally.All but one facilities was established over 20 years ago, and each cares for over 1500 patients. Hospital admissions ranged from 100 to 660 patients/unit/year. Of the total number of registered patients, 52-81% had undergone one or more reparative surgeries. Reoperations constituted 25-80% of the 50-170 operations/unit/year. Overall mean surgical mortality was 1.9%/year. Inpatient and outpatient care was provided in adult (n = 4) or both adult and pediatric (n = 2) settings. All six facilities enjoyed close collaboration between adult and pediatric cardiologists, cardiac surgeons, nurse specialists and cardiac and non-cardiac consultants. Training and research were pivotal activities.Provision of comprehensive care by multidisciplinary teams including adult and pediatric cardiologists, cardiac surgeons, specialized nurses and other cardiac and non-cardiac consultants was the unifying feature for all six tertiary care facilities reported here. There were minor differences among them based on available resources, local expertise and national health care policies. There appears to be a significant shortfall in tertiary care provision for the adult with CHD that requires further planning and resource allocation. These data may be useful for new and evolving adult CHD services.

  View details for DOI 10.1016/ijcard.2003.06.019

  View details for Web of Science ID 000223117000014

  View details for PubMedID 15262035

• Pediatric cardiology and adult congenital heart disease 53rd Annual Scientific Session of the American-College-of-Cardiology Murphy, D. J. ELSEVIER SCIENCE INC. 2004: 23A–24A

  View details for DOI 10.1016/j.jacc.2004.06.019

  View details for Web of Science ID 000222914200008

  View details for PubMedID 15261687

• Comparison of transthoracic echocardiography versus cardiovascular magnetic resonance imaging for the assessment of ventricular function in adults after atrial switch procedures for complete transposition of the great arteries AMERICAN JOURNAL OF CARDIOLOGY Lissin, L. W., Li, W., Murphy, D. J., Hornung, T., Swan, L., Mullen, M., Kilner, P., Gatzoulis, M. A. 2004; 93 (5): 654-657

  Abstract

  Adult patients with a history of transposition of the great arteries and atrial switch operation were studied with transthoracic echocardiography and cardiovascular magnetic resonance imaging. Measurements of ventricular dimensions and function by these 2 techniques in 18 consecutive patients were well correlated.

  View details for DOI 10.1016/j.amjcard.2003.11.044

  View details for Web of Science ID 000220075800033

  View details for PubMedID 14996604

• Pediatric cardiology and adult congenital heart disease. Journal of the American College of Cardiology Murphy, D. J. 2003; 42 (2): 380-381

  View details for PubMedID 12875781

• Simplified technique for correction of anomalous origin of left coronary artery from the anterior aortic sinus ANNALS OF THORACIC SURGERY Karamichalis, J. M., Vricella, L. A., Murphy, D. J., Reitz, B. A. 2003; 76 (1): 266-267

  Abstract

  Anomalous origin of the left main coronary artery from the right anterior coronary sinus has been associated with high incidence of sudden death in young adults. We describe a simplified approach to this rare congenital anomaly, which avoids the need for commissural post resuspension or relocation of the coronary button.

  View details for Web of Science ID 000183968400059

  View details for PubMedID 12842554

• Comparison of transthoracic echocardiography and cardiac magnetic resonance imaging for biventricular function in adult patients after the atrial switch procedure 52nd Annual Scientific Session of the American-College-of-Cardiology Lissin, L. W., Li, W., Hornung, T., Swan, L., Murphy, D. J., Mullen, M., Kilner, P., Gatzoulis, M. A. ELSEVIER SCIENCE INC. 2003: 491A–491A

  View details for Web of Science ID 000181669502127