Evolving nutritional needs, obesity, and overweight status in cystic fibrosis.

Purpose Of Review: The nutritional landscape in cystic fibrosis has shifted dramatically in the era of CFTR modulator therapy. In this review, we will critically examine the literature on overweight and obesity in CF, current nutritional care unknowns and opportunities for further investigation or adaptation in clinical care.

Recent Findings: Results of clinical trial and real-world data reflect marked improvement in nutritional status and quality of life.

Experiences of cystic fibrosis newborn screening and genetic counseling.

The South Carolina cystic fibrosis (CF) newborn screening (NBS) program changed in 2019 to include CFTR genotyping for babies with top 4% immunoreactive trypsinogen, which improves sensitivity and timeliness but increases carrier detection. Carrier identification has genetic implications for the family and parents of NBS+ babies have increased emotional distress. Genetic counseling (GC) may increase parent understanding and reduce anxiety yet is not uniformly offered at CF centers.

A case report of CFTR modulator administration via carrier mother to treat meconium ileus in a F508del homozygous fetus.

We report elexacaftor-tezacaftor-ivacaftor (ETI) treatment of a F508del carrier who was pregnant with a F508del homozygous fetus. At 23-weeks gestation meconium ileus (MI) was evident on ultrasound including dilated, hyperechoic bowel, which persisted on subsequent imaging. Through shared decision-making, the mother began ETI at 32 weeks with intent to treat fetal MI.

Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry.

Strong emphasis has been placed historically on increasing weight and improving nutritional status in cystic fibrosis patients. Due to correlation between nutritional indices (e.g.

Optimizing outcomes of pulmonary exacerbations in cystic fibrosis.

Purpose Of Review: Pulmonary exacerbations are described as worsening of the daily symptoms of cystic fibrosis airways disease, typically with increased cough and sputum production. There are often associated signs such as weight loss and reduced lung function. These events occur frequently and are associated with considerable cost and morbidity.

Combined effects of multiple risk factors on asthma in school-aged children.

Background: Little is known about synergistic effects of several risk factors on asthma. We developed a risk score in Puerto Rican children, and then used this score to estimate the combined effects of multiple risk factors on asthma at school age in Puerto Rican and Swedish children.

Methods: Case-control study in 609 Puerto Rican children (aged 6-14 years) and longitudinal birth cohort study of 2290 Swedish children followed up to age 12 years (The Children, Allergy, Milieu, Stockholm, Epidemiological Survey [BAMSE] Study).

Clinical correlates of lung ventilation defects in asthmatic children.

Background: Lung ventilation defects identified by using hyperpolarized 3-helium gas ((3)He) lung magnetic resonance imaging (MRI) are prevalent in asthmatic patients, but the clinical importance of ventilation defects is poorly understood.

Objectives: We sought to correlate the lung defect volume quantified by using (3)He MRI with clinical features in children with mild and severe asthma.

Methods: Thirty-one children with asthma (median age, 10 years; age range, 3-17 years) underwent detailed characterization and (3)He lung MRI.

Chest pain and ventricular tachycardia in a patient with surgically corrected anomalous right coronary artery from the left sinus of Valsalva.

We present a case of an adult patient who had anomalous origin of the right coronary artery (RCA) from the left sinus of Valsalva that had been treated surgically in the past and who presented years later with chest pain and runs of nonsustained ventricular tachycardia. Coronary angiography showed a patent unroofed RCA with appropriately repositioned origin and no obstructive coronary artery disease. This case presents angiographic documentation of a technically satisfactory repair of anomalous aortic origin of a coronary artery and suggests that potentially lethal arrhythmia can occur despite a technically satisfactory repair.

The safety of preoperative vardenafil in patients undergoing coronary artery bypass graft surgery.

Phosphodiesterase 5 inhibitors are cardioprotective against myocardial reperfusion ischemic injury in animal models but are contraindicated in patients with coronary disease who take nitrates because of a risk for hypotension. We investigated the safety of vardenafil in patients undergoing coronary artery bypass grafting (CABG) surgery. A single dose of vardenafil was given to 10 patients before CABG surgery.

Beware of the B(e)all valve: mistaken valve identity, 30-year survival, and valve replacement.

Effective management of patients after the implantation of mechanical cardiac valves includes correct recognition of each valve and its related complications. Herein, we present the case of a patient who had undergone implantation of a floating-disc Beall-Surgitool mitral valve in 1976 and developed multiple valve-related complications. Over 30 years and in multiple medical centers, the device was mistakenly assumed to be a "ball" valve.

North American multicenter experience with the On-X prosthetic heart valve.

Background And Aim Of The Study: This ongoing, longitudinal, multi-center, North American study was designed to evaluate the safety and effectiveness of the On-X valve.

Methods: The On-X valve was implanted in isolated aortic (AVR) and mitral (MVR) valve replacement patients at nine North American centers. Follow up was 98.

Adjunctive transmyocardial revascularization: five-year follow-up of a prospective, randomized trial.

Background: In a prospective, randomized trial involving 263 patients who would be incompletely revascularized by coronary artery bypass grafting (CABG) alone, CABG plus transmyocardial revascularization (CABG/TMR) provided an early mortality benefit with similar angina relief compared with CABG alone at 1 year. We evaluated the long-term outcome of patients randomized to CABG/TMR or CABG alone.

Methods: Thirteen centers that enrolled 83% (218/263) of the patients in the original trial participated in this longitudinal study.

Transmyocardial laser revascularization combined with coronary artery bypass grafting: a multicenter, blinded, prospective, randomized, controlled trial.

Objective: We sought to assess the safety and efficacy of transmyocardial revascularization combined with coronary artery bypass grafting in patients not amenable to complete revascularization by coronary bypass alone.

Methods: A total of 263 patients whose standard of care was coronary artery bypass grafting and who had one or more ischemic areas not amenable to bypass grafting were prospectively randomized to receive coronary bypass of suitable vessels plus transmyocardial revascularization to areas not graftable (n = 132) or coronary bypass alone with nongraftable areas left unrevascularized (n = 131). Group preoperative demographics and operative characteristics were similar.

Mitral valve repair combined with aortic valve replacement.

Background And Aims Of The Study: Combined aortic and mitral valve replacement continues to result in significant morbidity and mortality. Although mitral repair has improved the results of mitral valve surgery, its influence on combined aortic valve replacement has not been assessed.

Methods: We reviewed 38 consecutive patients who underwent aortic valve replacement (AVR) and mitral repair (MR) between 1985 and 1995.

Sensing lead-related complications in patients with transvenous implantable cardioverter-defibrillators.

The widespread use of the redesigned Endotak lead (CPI, St. Paul, Minnesota), which combines transvenous pacing, sensing, and defibrillation on a single transvenous lead in patients receiving transvenous implantable cardioverter-defibrillators (ICDs), has reduced morbidity and shortened length of hospital stay after ICD implantation. We describe the incidence and management of Endotak sensing lead-related failures in a series of 348 consecutive patients from 4 institutions who underwent implantation between 1990 and 1995.

Mitral valve repair in patients over the age of 70 years.

The question of whether to repair or replace the mitral valve in the elderly remains unanswered. The purpose of our study is to describe our experience with mitral valve repair (MVR) using Carpentier's technique in patients 70 years and older. Fifty consecutive patients underwent MVR between 1984-1992.

Deep hypothermic arrest and left thoracotomy for repair of difficult thoracic aneurysms.

Seven patients with complex thoracic aortic aneurysms were operated on using profound hypothermia and circulatory arrest through a left thoracotomy. Three patients had false aneurysms, 2 had large aneurysms precluding access for proximal control, 1 patient had had previous hemiarch replacement, and 1 patient had a thoracoabdominal aneurysm. All patients were cooled on partial cardiopulmonary bypass until the electroencephalogram was isoelectric (approximately 15 degrees C rectal temperature).

Management of aortic cannulation site blowouts.

An alternative technique of handling the aortic cannulation site blowout is described. The cannulation site is excluded with a side-biting clamp. The area is excised.

A prospective study of the clinical utility of lymphocyte monitoring in the cardiac transplant recipient.

Monitoring peripheral lymphocytes for changes in antigen expression or subpopulations was performed by flow cytometry in an attempt to identify infection or rejection in cardiac transplantation recipients (CTRs). In this study, 32 cardiac transplantation recipients were followed prospectively, and the results of 274 lymphocyte analyses for transferrin receptor expression, an indicator of lymphocyte activation, and CD4/CD8 lymphocyte ratios were correlated with the patient's clinical status, e.g.

Symptomatic abdominal aortic aneurysms in long-term survivors of cardiac transplantation.

Herein we report the only two long-term survivors of cardiac transplantation who underwent successful repair of symptomatic abdominal aortic aneurysms since the advent of cyclosporine therapy in 1983. Review of the world's literature indicates that the only two recorded cases of repair of symptomatic abdominal aortic aneurysms after cardiac transplantation occurred before the use of cyclosporine. The presentation and clinical course of our patients recently treated are presented, and perioperative care and immunosuppressive management are outlined.

Subnormal heart period variability in heart failure: effect of cardiac transplantation.

Heart period variability and arterial baroreceptor-cardiac reflex function were studied in cardiac transplant patients to determine if correction of heart failure restores parasympathetic control mechanisms toward normal. Heart period variability (standard deviation [SD] of 120 consecutive RR or PP intervals) was measured at supine rest in 34 patients with congestive heart failure (23 patients receiving diuretics, digoxin or vasodilators and 11 patients weaned from all medications), 30 cardiac transplant patients (both innervated recipient and denervated donor atrial rates) and 16 age-matched healthy control subjects. Arterial baroreflex gain was evaluated with intravenous bolus injections of phenylephrine in 22 transplant patients.