FFRCT: Current Status.

OBJECTIVE. Fractional flow reserve derived from coronary CT angiography (FFRCT) is an emerging tool for noninvasive evaluation of coronary artery disease that provides a combined anatomic and physiologic evaluation. The goal of this article is to serve as a review of the current status of FFRCT through discussion of existing trials on the modality and to introduce readers to examples of its utility and potential pitfalls. CONCLUSION. This article reviews the current body of evidence on FFRCT and provides case examples illustrating its current uses, limitations, and potential future applications.

Subclinical involvement of common carotid arteries in patients with fibromuscular dysplasia - a case-control study.

Background: Fibromuscular dysplasia (FMD) primarily involves medium-sized arteries, though the entire spectrum of vascular involvement is not fully understood. We hypothesized that larger arteries may also be affected, albeit sub-clinically. Patients and methods: We measured the cross-sectional diameter of the thoracic aorta, abdominal aorta, common iliac arteries (CIA) and common carotid arteries (CCA) in FMD subjects and compared them to matched controls. We retrospectively analyzed records of FMD subjects (n = 74) and of age- and sex- matched controls (n = 74) that underwent computed tomography of the neck, chest or abdomen. Cross-sectional diameters of the thoracic and abdominal aorta, CIA and CCA were measured in a standardized manner by two trained physicians. Results: The FMD group had a significantly greater diameter of the CIA and CCA bilaterally. The measurements (mm) in FMD and control groups were as follows: Right CIA: 10.85 + 1.75 vs. 10.23 + 1.36, p = 0.04, left CIA: 11.01 + 1.93 vs. 10.15 + 1.38, p = 0.007, right CCA: 7.70 + 0.81 vs. 6.80 + 1.10, p < 0.001 and left CCA: 7.70 + 1.10 vs. 6.80 + 1.0, p < 0.001). There was no difference in the diameter between the two groups in the ascending aorta, descending and the abdominal aorta. After adjusting for baseline differences, common carotid arteries (but not common iliac) were significantly larger in FMD group compared with controls. Conclusions: There is sub-clinical involvement of the common carotid arteries in patients with FMD and this manifests as a greater diameter of these arteries compared to age and sex matched controls.

Metastatic Adenocarcinoma of Intestinal Origin in Reconstructed Ureters.

In patients with long ureteral defects, the use of bowel segments for reconstruction is an effective but suboptimal alternative because the bowel is not resistant to the potential carcinogenic effects of urine. Primary malignancies in reconstructed conduits have been scarcely described in the literature. This case report elaborates on a case of metastatic adenocarcinoma arising in ureters reconstructed using small intestinal segments. A 49-year-old with Eagle-Barrett syndrome presented with abdominal pain and was found to have irregular enhancement of the right ureteropelvic junction and small, non-specific liver lesions. Biopsy of the liver lesions showed poorly differentiated adenocarcinoma with immunohistochemistry staining consistent with small intestinal origin. The patient was treated as a tumor of GI origin with chemotherapy and subsequently underwent microwave ablation of his liver metastases. He also received concurrent chemoradiation for residual disease at the ureteral conduit. PET scan images done after completion of treatment showed resolution of all lesions. Further research into alternative structures that could be used to create conduits and screening methods for these patients is imperative to reduce the incidence of such malignancies.

Analysis of baseline parameters in the HALT polycystic kidney disease trials.

HALT PKD consists of two ongoing randomized trials with the largest cohort of systematically studied patients with autosomal dominant polycystic kidney disease to date. Study A will compare combined treatment with an angiotensin-converting inhibitor and receptor blocker to inhibitor alone and standard compared with low blood pressure targets in 558 early-stage disease patients with an eGFR over 60 ml/min per 1.73 m(2). Study B will compare inhibitor-blocker treatment to the inhibitor alone in 486 late-stage patients with eGFR 25-60 ml/min per 1.73 m(2). We used correlation and multiple regression cross-sectional analyses to determine associations of baseline parameters with total kidney, liver, or liver cyst volumes measured by MRI in Study A and eGFR in both studies. Lower eGFR and higher natural log-transformed urine albumin excretion were independently associated with a larger natural log-transformed total kidney volume adjusted for height (ln(HtTKV)). Higher body surface area was independently associated with a higher ln(HtTKV) and lower eGFR. Men had larger height-adjusted total kidney volume and smaller liver cyst volumes than women. A weak correlation was found between the ln(HtTKV) and natural log-transformed total liver volume adjusted for height or natural log liver cyst volume in women only. Women had higher urine aldosterone excretion and lower plasma potassium. Thus, our analysis (1) confirms a strong association between renal volume and functional parameters, (2) shows that gender and other factors differentially affect the development of polycystic disease in the kidney and liver, and (3) suggests an association between anthropomorphic measures reflecting prenatal and/or postnatal growth and disease severity.

Use of brain MRI after deep brain stimulation hardware implantation.

The objective of this study was to examine the experience with and safety of brain 1.5 Tesla (T) magnetic resonance imaging (MRI) in deep brain stimulation (DBS) patients. This was a retrospective review of brain MRI scanning performed on DBS patients at the University of Kansas Medical Center between January 1995 and December 2007. A total of 249 DBS patients underwent 445 brain 1.5 T MRI scan sessions encompassing 1,092 individual scans using a transmit-receive head coil, representing the cumulative scanning of 1,649 DBS leads. Patients with complete implanted DBS systems as well as those with externalized leads underwent brain imaging. For the majority of scans, specific absorption rates localized to the head (SAR(H)) were estimated and in all cases SAR(H) were higher than that specified in the present product labeling. There were no clinical or hardware related adverse events secondary to brain MRI scanning. Our data should not be extrapolated to encourage MRI scanning beyond the present labeling. Rather, our data may contribute to further defining safe MRI scanning parameters that might ultimately be adopted in future product labeling as more centers report in detail their experiences.

Volume progression in polycystic kidney disease.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive enlargement of cyst-filled kidneys. METHODS: In a three-year study, we measured the rates of change in total kidney volume, total cyst volume, and iothalamate clearance in patients with ADPKD. Of a total of 241 patients, in 232 patients without azotemia who were 15 to 46 years old at baseline we used magnetic-resonance imaging to correlate the total kidney volume and total cyst volume with iothalamate clearance. Statistical methods included analysis of variance, Pearson correlation, and multivariate regression analysis. RESULTS: Total kidney volume and total cyst volume increased exponentially, a result consistent with an expansion process dependent on growth. The mean (+/-SD) total kidney volume was 1060+/-642 ml at baseline and increased by a mean of 204+/-246 ml (5.27+/-3.92 percent per year, P<0.001) over a three-year period among 214 patients. Total cyst volume increased by 218+/-263 ml (P<0.001) during the same period among 210 patients. The baseline total kidney volume predicted the subsequent rate of increase in volume, independently of age. A baseline total kidney volume above 1500 ml in 51 patients was associated with a declining glomerular filtration rate (by 4.33+/-8.07 ml per minute per year, P<0.001). Total kidney volume increased more in 135 patients with PKD1 mutations (by 245+/-268 ml) than in 28 patients with PKD2 mutations (by 136+/-100 ml, P=0.03). CONCLUSIONS: Kidney enlargement resulting from the expansion of cysts in patients with ADPKD is continuous and quantifiable and is associated with the decline of renal function. Higher rates of kidney enlargement are associated with a more rapid decrease in renal function.

Imminent cardiac risk assessment via optical intravascular biochemical analysis.

Heart disease is by far the biggest killer in the United States, and type II diabetes, which affects 8% of the U.S. population, is on the rise. In many cases, the acute coronary syndrome and/or sudden cardiac death occurs without warning. Atherosclerosis has known behavioral, genetic and dietary risk factors. However, our laboratory studies with animal models and human post-mortem tissue using FT-IR microspectroscopy reveal the chemical microstructure within arteries and in the arterial walls themselves. These include spectra obtained from the aortas of ApoE-/- knockout mice on sucrose and normal diets showing lipid deposition in the former case. Also pre-aneurysm chemical images of knockout mouse aorta walls, and spectra of plaque excised from a living human patient are shown for comparison. In keeping with the theme of the SPEC 2008 conference 'Spectroscopic Diagnosis of Disease...' this paper describes the background and potential value of a new catheter-based system to provide in vivo biochemical analysis of plaque in human coronary arteries. We report the following: (1) results of FT-IR microspectroscopy on animal models of vascular disease to illustrate the localized chemical distinctions between pathological and normal tissue, (2) current diagnostic techniques used for risk assessment of patients with potential unstable coronary syndromes, and (3) the advantages and limitations of each of these techniques illustrated with patent care histories, related in the first person, by the physician coauthors. Note that the physician comments clarify the contribution of each diagnostic technique to imminent cardiac risk assessment in a clinical setting, leading to the appreciation of what localized intravascular chemical analysis can contribute as an add-on diagnostic tool. The quality of medical imaging has improved dramatically since the turn of the century. Among clinical non-invasive diagnostic tools, laboratory tests of body fluids, EKG, and physical examination are still the first line of defense. However, with the fidelity of 64-slice CT imaging, this technique has recently become an option when the patient presents with symptoms of reduced arterial flow. Single photon emission computerized tomography (SPECT) treadmill exercise testing is a standard non-invasive test for decreased perfusion of heart muscle, but is time consuming and not suited for emergent evaluation. Once the invasive clinical option of catherization is chosen, this provides the opportunity for intravascular ultrasound (IVUS) imaging. As the probe is pulled through the artery, the diameter at different parts is measurable, and monochrome contrast in the constricted area reveals the presence of tissue with a different ultrasonic response. Also, via an optical catheter with a fiber-optic conductor, the possibly of spectroscopic analysis of arterial walls is now a reality. In this case, the optical transducer is coupled to a near-infrared spectrometer. Revealing the arterial chemical health means that plaque vulnerability and imminent risk could be assessed by the physician. The classical emergency use of catherization involves a contrast agent and dynamic X-ray imaging to locate the constriction, determine its severity, and possibly perform angioplasty, and stent placement.

Myocarditis Causing Premature Ventricular Contractions: Insights From the MAVERIC Registry.

BACKGROUND: Premature ventricular contractions are a common clinical presentation that drives further diagnostic workup. We hypothesize the presence of underlying inflammation is often unrecognized in these patients with a potential for continued disease progression if not diagnosed and treated early in the disease course. METHODS: This is a single-center, prospective study including 107 patients with frequent symptomatic premature ventricular contractions (>5000/24 h) and no known ischemic heart disease. Patients underwent a combination of laboratory testing, 18F-fluorodeoxyglucose positron emission tomography scan, cardiac magnetic resonance imaging, and biopsy. Patients were diagnosed with myocarditis based on a multidisciplinary approach and treated with immunosuppressive therapy. RESULTS: The mean age of the cohort was 57±15 years, 41% were males, and left ventricular ejection fraction was 47±11.8%. Positive positron emission tomography scan was seen in 51% (55/107), of which 51% (28/55) had preserved left ventricle function. Based on clinical profile, 18F-fluorodeoxyglucose-positron emission tomography imaging, cardiac magnetic resonance, and histological data 58% patients (32/55) received immunosuppressive therapy alone and 25.4% (14/55) received immunosuppressive therapy and catheter ablation. Optimal response was seen in 67% (31/46) over a mean follow-up of 6±3 months. In patients with left ventricle systolic dysfunction, 37% (10/27) showed an improvement in mean left ventricular ejection fraction of 13±6%. CONCLUSIONS: Approximately 51% of patients presenting with frequent premature ventricular contractions have underlying myocardial inflammation in this cohort. 18F-fluorodeoxyglucose-positron emission tomography scan can be a useful modality for early diagnosis and treatment with immunosuppressive therapy in selected patients can improve clinical outcomes.

Disseminated cat-scratch disease in an adult with selective IgA deficiency.

A 51-year-old man with history of undiagnosed pulmonary nodules 4 years prior, presented with right-sided chest pain. Acute cardiac workup was negative, and a chest computed tomography examination demonstrated marked improvement in bilateral pulmonary nodules. A concordant abdominal computed tomography examination showed new subcentimeter hypodense lesions throughout the liver and spleen, mild progressive abdominopelvic lymphadenopathy, and new small lytic lesions of T11 and L4 vertebrae. A positron emission tomography examination demonstrated hypermetabolic activity of these abdominopelvic lesions suggesting metastatic disease. Extensive laboratory workup was negative, aside from IgA deficiency. Eventually, biopsy of a hepatic lesion was performed and compatible with Bartonella species. An elevated Bartonella IgG titer was noted, consistent with Bartonella Hensalae infection, or "cat-scratch disease." Radiographic findings showed marked improvement after clinically appropriate antibiotic therapy.

Sonographic assessment of the severity and progression of autosomal dominant polycystic kidney disease: the Consortium of Renal Imaging Studies in Polycystic Kidney Disease (CRISP).

BACKGROUND: The accuracy and precision of ultrasonography (US) in assessing the severity of autosomal dominant polycystic kidney disease (ADPKD) is unknown. METHODS: US and magnetic resonance imaging (MRI) were performed at baseline and 1 year on 230 subjects with ADPKD. Ellipsoid volume was calculated from US length, width, and depth, and sequential transverse images were used to measure total and cystic volume directly. These were compared with MRI measurements of kidney volume and cystic volume. RESULTS: Variability between different sonographers ranged from 18% to 42%. Correlations between US and MRI volume were 0.88 and 0.89. The SD of the discrepancy from MRI ranged from 21% to 33% and was unrelated to kidney size or body mass. Kidney length was the most reproducible measurement, and its correlation with MRI volume was 0.84. All patients with an US volume less than 700 cm3 had an MRI volume less than 1,000 cm3, and all patients with an US volume greater than 1,700 cm3 had an MRI volume greater than 1,000 cm3. Increases in volume after 1 year were 12% +/- 36% for the ellipsoid method, 6% +/- 29% for the direct method, and 4.2% +/- 7.2% for MRI. Correlation between US and MRI measurement of fractional cyst volume was 0.80. CONCLUSION: Sonographic measurement of kidney volume in patients with ADPKD is inaccurate and lacks the precision necessary to measure short-term disease progression. However, sonography can provide an estimate of kidney volume that reflects severity and prognosis in individual patients.

Effect of distance and infusion rate on operation of Medfusion 2010 infusion pump during magnetic resonance imaging.

STUDY OBJECTIVES: To determine the accuracy and reliability of intravenous infusion, as well as magnetic resonance image effect of the Medfusion 2010 infusion pump (Medex Medical Supplies, Inc., Duluth, GA) at distances of 2, 4, 8, 12, and 16 feet from a 1.5 Tesla MRI magnet over a four-week time interval, using infusion rates that would correspond to those of propofol administration. DESIGN: Prospective, open-label study. SETTING: Radiology department MRI in an American academic medical center. INTERVENTIONS: Five infusion pumps, including one outside the MRI suite as control, were tested. Pumps were evaluated at distances of 2, 4, 8, 12, and 16 feet from a 1.5 Tesla magnet. One pump at distances of 2 and 16 feet was tested during 30 and 90 hours of operation, respectively. Three pumps at distances of 4, 8, and 12 feet were tested during 120 hours of operation. Pump batteries were recharged for 18 hours outside the MRI suite between the 6-hour test periods. Distilled, deionized water was infused from a 30-mL or 60-mL syringe via a 36-inch Medex small-bore extension set into a graduated collection cylinder at rates of 5, 10.5, 21, or 42 mL/hr. Each rate was increased to the next infusion rate level at 30-hour weekly intervals. The collection cylinder with infused water was weighed on an electronic scale accurate to within +/-0.001 g. Analysis of variance and regression analysis were used to analyze data. A p < 0.05 value was considered statistically significant. MEASUREMENTS AND MAIN RESULTS: There was no significant difference comparing the grams weight of volume measured and the mL volume visualized within and between groups at varying distances from the magnet. Increasing infusion rates resulted in corresponding increases in volume delivered irrespective of the distance from the magnet. CONCLUSIONS: The Medfusion 2010 infusion pumps were found to be reliable and accurate, without causing failure or any significant degradation of MRI images compared to control at infusion rate of 42 mL/hr at a distance of 2 feet for 30 hours; and a rate of 5.0, 10.5, 21, and 42 mL/hr at 16 feet for 90 hours; and at 4, 8, and 12 feet for 120 hours of operation.

Inflammatory Myofibroblastic Tumor: A Case Study.

Inflammatory myofibroblastic tumor (IMT) is a rare benign lesion found in many locations throughout the body and genitourinary tract. Endoscopically and radiographically, these solid lesions cannot be distinguished from malignant bladder tumors. We present the case of a 21-year-old woman who presented with painful obstructive and irritative voiding symptoms of short duration. After extensive preoperative evaluation failed to reveal a definitive diagnosis, the patient underwent partial cystectomy. Final pathology revealed IMT. A high index of suspicion is required for diagnosis of IMT as it is often difficult to distinguish from its malignant counterparts.

Anterior Spinal Surgery Involving Variant Vascular Anatomy: A Case Report.

STUDY DESIGN: Case report of an anterior approach to the spine in the setting of variant vascular anatomy. OBJECTIVE: To highlight the importance of evaluating vascular anatomy before anterior lumbar spine surgery. SUMMARY OF BACKGROUND DATA: A 62-year-old woman with idiopathic scoliosis had thoracolumbar fusion in adolescence and subsequently developed symptomatic sub-adjacent segment breakdown. Vascular complications may be encountered during anterior approaches to the spine. Variation in vascular anatomy may compound the difficulty of an already meticulous dissection. RESULTS: A patient with idiopathic scoliosis who had thoracolumbar fusion in adolescence and subsequently developed symptomatic sub-adjacent segment breakdown. She underwent a 2-stage posterior/anterior procedure. During the anterior retroperitoneal approach, an anomalous left inferior vena cava was encountered that required tedious dissection for safe and adequate exposure of the lumbar spine. CONCLUSIONS: When planning anterior lumbar spine surgery, careful review of the vascular anatomy on imaging should be performed. This will help prepare the surgeon for more complex or anomalous anterior anatomy. If atypical vascular anatomy is identified, consideration of a pathologic cause should be investigated.

Detected renal cysts are tips of the iceberg in adults with ADPKD.

BACKGROUND AND OBJECTIVES: In autosomal dominant polycystic kidney disease, progressive renal enlargement secondary to expanding cysts is a hallmark. The total cyst load and range of cyst diameters are unknown. The purpose of this study was to quantify the total number and range of diameters of individual cysts in adults with preserved GFR. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: A retrospective, morphometric analysis of renal cyst number and diameter using magnetic resonance images from eight adult autosomal dominant polycystic kidney disease patients was performed at baseline and after 6.9 years. Cyst number and diameter were measured in microscopic sections of nephrectomy specimens from five different adults. RESULTS: The diameters of 1010 cysts ranged from 0.9 to 77.1 mm in baseline T2 magnetic resonance images, and the mean total number of cysts increased from 682 to 1002 in 6.9 years. However, magnetic resonance imaging detects only cysts above the lower limit of detection. In 405 cysts measured in nephrectomy specimens, 70% had diameters <0.9 mm. Cyst counts by magnetic resonance in eight subjects compared with histology revealed approximately 62 times more cysts below the limit of magnetic resonance imaging detection than above it. CONCLUSIONS: This study presents quantitative data indicating that renal cysts develop in a minority of renal tubules. Increased numbers detected by magnetic resonance imaging are caused primarily by cysts below detection at baseline enlarging to a detectable diameter over time. The broad range of diameters, with a heavy concentration of microscopic cysts, may be most appropriately explained by a formation process that operates continuously throughout life.

Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease.

BACKGROUND AND OBJECTIVES: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by increased total kidney volume (TKV) and renal failure. This study aimed to determine if height-adjusted TKV (htTKV) predicts the onset of renal insufficiency. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This prospective, observational, longitudinal, multicenter study included 241 adults with ADPKD and preserved renal function. Magnetic resonance imaging and iothalamate clearance were used to measure htTKV and GFR, respectively. The association between baseline htTKV and the attainment of stage 3 CKD (GFR <60 ml/min per 1.73 m(2)) during follow-up was determined. RESULTS: After a mean follow-up of 7.9 years, stage 3 CKD was attained in 30.7% of the enrollees. Using baseline htTKV, negative correlations with GFR increased from -0.22 at baseline to -0.65 at year 8. In multivariable analysis, a baseline htTKV increase of 100 cc/m significantly predicted the development of CKD within 8 years with an odds ratio of 1.48 (95% confidence interval: 1.29, 1.70). In receiver operator characteristic curve analysis, baseline htTKV of 600 cc/m most accurately defined the risk of developing stage 3 CKD within 8 years with an area under the curve of 0.84 (95% confidence interval: 0.79, 0.90). htTKV was a better predictor than baseline age, serum creatinine, BUN, urinary albumin, or monocyte chemotactic protein-1 excretion (P<0.05). CONCLUSIONS: Baseline htTKV ≥600 cc/m predicted the risk of developing renal insufficiency in ADPKD patients at high risk for renal disease progression within 8 years of follow-up, qualifying htTKV as a prognostic biomarker in ADPKD.

Potentially modifiable factors affecting the progression of autosomal dominant polycystic kidney disease.

BACKGROUND AND OBJECTIVES: The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) was created to identify markers of disease progression in patients with autosomal dominant polycystic kidney disease (ADPKD). DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Linear mixed models were utilized to model effects of baseline parameters on changes in natural-log (ln)-transformed total kidney volume (TKV) and iothalamate clearance (GFR) across time in CRISP participants (creatinine clearance at entry >70 ml/min). Stepwise selection was used to obtain a final main effect model. RESULTS: TKV increased from year to year, whereas GFR uncorrected for body surface area (BSA) decreased only at year 6. Higher lnTKV and urine sodium excretion (U(Na)V), lower serum HDL-cholesterol, and younger age at baseline associated with greater lnTKV growth from baseline to year 3 and to year 6. Higher lnTKV at baseline associated with greater GFR decline from year 1 to year 3 and to year 6. Higher BSA and 24-hour urine osmolality at baseline associated with greater GFR decline from year 1 to year 6. Higher U(Na)V and lower serum HDL-cholesterol at baseline associated with greater GFR decline from year 1 to year 6 by univariate analysis only. Associations seen during year 1 to year 6 (not seen during year 1 to year 3) reflect the time lag between structural and functional disease progression. CONCLUSIONS: Serum HDL-cholesterol, U(Na)V, and 24-hour urine osmolality likely affect ADPKD progression. To what extent their modification may influence the clinical course of ADPKD remains to be determined.

Cardiac magnetic resonance assessment of left ventricular mass in autosomal dominant polycystic kidney disease.

BACKGROUND AND OBJECTIVES: Autosomal dominant polycystic kidney disease (ADPKD) is associated with a substantial cardiovascular disease burden including early onset hypertension, intracranial aneurysms, and left ventricular hypertrophy (LVH). A 41% prevalence of LVH has been reported in ADPKD, using echocardiographic assessment of LV mass (LVM). The HALT PKD study was designed to assess the effect of intensive angiotensin blockade on progression of total kidney volume and LVM. Measurements of LVM were performed using cardiac magnetic resonance (MR). DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Five hundred forty-three hypertensive patients with GFR >60 ml/min per 1.73 m(2) underwent MR assessment of LVM at baseline. LVM was adjusted for body surface area and expressed as LVM index (LVMI; g/m(2)). RESULTS: Baseline BP was 125.1 ± 14.5/79.3 ± 11.6 mmHg. Average duration of hypertension was 5.79 years. Prior use of angiotensin-converting enzyme inhibitors or angiotensin receptor blockers was present in 59.5% of patients. The prevalence of LVH assessed using nonindexed LVM (g) was 3.9% (n = 21, eight men and 13 women) and 0.93% (n = 5, one man and four women) using LVMI (g/m(2)). In exploratory analyses, the prevalence of LVH using LVM indexed to H(2.7), and the allometric index ppLVmass(HW), ranged from 0.74% to 2.23% (n = 4 to 12). Multivariate regression showed significant direct associations of LVMI with systolic BP, serum creatinine, and albuminuria; significant inverse associations with LVMI were found with age and female gender. CONCLUSIONS: The prevalence of LVH in hypertensive ADPKD patients <50 years of age with short duration of hypertension, and prior use of angiotensin-converting enzyme inhibitors/angiotensin receptor blockers is low. Early BP intervention in ADPKD may have decreased LVH and may potentially decrease cardiovascular mortality.

Evidence of extraordinary growth in the progressive enlargement of renal cysts.

BACKGROUND AND OBJECTIVES: In autosomal dominant polycystic kidney disease, cysts derived from tubules are detected at birth by ultrasound (threshold for detection >7.0 mm); thus, fetal cyst growth rates must exceed 2300%/yr. In adults, the combined renal cyst component enlarges at approximately 12%/yr by growth of individual cysts. To explore this discrepancy, the growth rates of individual cysts were determined in adult polycystic kidneys. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Diameter, volume, and growth rates of individual cysts were measured by magnetic resonance in 30 individual cysts in three adult patients over a span of 3 years. Results were confirmed in 22 cysts measured in five patients by computed tomography over a span of 11 years. RESULTS: Mean cyst diameters were 20.4 +/- 9.9 mm (range 7.1 to 40.5 mm) at baseline and 25.8 +/- 15.6 mm (range 7.8 to 49.6 mm) after 3 years. Mean cyst volumes, determined by manual segmentation and summation of magnetic resonance cross sections, were 8.7 +/- 12.9 cm(3) (0.3 to 43.3 cm(3)) and 24.2 +/- 66.3 cm(3) (0.3 to 364.8 cm(3)) after 3 years. Mean cyst growth rates ranged from 6.9 to 23.9%/yr; the maximum growth rate was 71.1%/yr, far less than required to develop a 7-mm diameter cyst in utero. Results were similar in 22 cysts examined by computed tomography. CONCLUSIONS: It was concluded that renal cysts detected by ultrasound in newborns must have grown at exuberant rates in utero; thereafter, expansion appears to proceed at much slower rates.

IntraCameral right coronary artery: detection by 64 slice coronary computed tomographic angiography and implications for radiofrequency ablation of atrial dysrhythmias.

An intracameral or intracavitary course for a coronary artery is a rare anomaly. Nevertheless, it carries a significant impact for invasive cardiac procedures that require right atrial catheterization, pacemaker implantation, or electrophysiologic study such as radiofrequency ablation. If a coronary artery were to be damaged within the atrial chamber by catheter manipulation at the time of heart catheterization, serious complications might ensue. We describe the first reported case of an intracameral right coronary artery identified with multidetector 64-slice coronary computed tomographic angiography performed prior to pulmonary venous antral isolation for atrial fibrillation.