Limits...
A Case of Idiopathic Hypereosinophilic Syndrome Causing Mitral Valve Papillary Muscle Rupture.

Tamse T, Rampersad A, Jordan-Villegas A, Ireland J - Case Rep Pediatr (2015)

Bottom Line: This disease can cause significant morbidity and mortality if left untreated.She was treated with steroids with improvement of her symptoms and scheduled for close follow-up.In general patients with IHES that have cardiac involvement have poorer prognoses.

View Article: PubMed Central - PubMed

Affiliation: Florida Hospital for Children, Orlando, FL 32803, USA ; University of Central Florida, Orlando, FL 32827, USA.

ABSTRACT
Idiopathic Hypereosinophilic Syndrome (IHES) is a rare disease that can be difficult to diagnose as the differential is broad. This disease can cause significant morbidity and mortality if left untreated. Our patient is a 17-year-old adolescent female who presented with nonspecific symptoms of abdominal pain and malaise. She was incidentally found to have hypereosinophilia of 16,000 on complete blood count and nonspecific colitis and pulmonary edema on computed tomography. She went into cardiogenic shock due to papillary rupture of her mitral valve requiring extreme life support measures including intubation and extracorporal membrane oxygenation (ECMO) as well as mitral valve replacement. Pathology of the valve showed eosinophilic infiltration as the underlying etiology. The patient was diagnosed with IHES after the exclusion of infectious, rheumatologic, and oncologic causes. She was treated with steroids with improvement of her symptoms and scheduled for close follow-up. In general patients with IHES that have cardiac involvement have poorer prognoses.

No MeSH data available.


Related in: MedlinePlus

(a) Chest X-ray showing complete opacification of the thorax suspicion for complete lung consolidation secondary to edema. (b) Echocardiogram images showing flail mitral valve from a ruptured cord and severe mitral regurgitation.
© Copyright Policy
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4660015&req=5

fig1: (a) Chest X-ray showing complete opacification of the thorax suspicion for complete lung consolidation secondary to edema. (b) Echocardiogram images showing flail mitral valve from a ruptured cord and severe mitral regurgitation.

Mentions: She continued to decompensate and was started on high frequency oscillatory ventilation with nitric oxide. Her chest X-ray showed worsening interstitial and alveolar edema (Figure 1(a)). At this time the patient went into multiorgan dysfunction syndrome, due to increased level of support, she was placed on VA ECMO. Cardiac echocardiography demonstrated severe mitral regurgitation from a nonfunctioning valve, moderate left atrial enlargement (Figure 1(b)). The patient was taken to the operating room for replacement of the mitral valve and intraoperatively there was noted to be papillary muscle avulsion of the anterior leaflet without evidence of vegetation. She was separated from ECMO support but developed acute renal failure requiring one week of hemodialysis. She was extubated on day ten of hospitalization and kidney function recovered completely. Neurologically she has remained without any deficits noted.


A Case of Idiopathic Hypereosinophilic Syndrome Causing Mitral Valve Papillary Muscle Rupture.

Tamse T, Rampersad A, Jordan-Villegas A, Ireland J - Case Rep Pediatr (2015)

(a) Chest X-ray showing complete opacification of the thorax suspicion for complete lung consolidation secondary to edema. (b) Echocardiogram images showing flail mitral valve from a ruptured cord and severe mitral regurgitation.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4660015&req=5

fig1: (a) Chest X-ray showing complete opacification of the thorax suspicion for complete lung consolidation secondary to edema. (b) Echocardiogram images showing flail mitral valve from a ruptured cord and severe mitral regurgitation.
Mentions: She continued to decompensate and was started on high frequency oscillatory ventilation with nitric oxide. Her chest X-ray showed worsening interstitial and alveolar edema (Figure 1(a)). At this time the patient went into multiorgan dysfunction syndrome, due to increased level of support, she was placed on VA ECMO. Cardiac echocardiography demonstrated severe mitral regurgitation from a nonfunctioning valve, moderate left atrial enlargement (Figure 1(b)). The patient was taken to the operating room for replacement of the mitral valve and intraoperatively there was noted to be papillary muscle avulsion of the anterior leaflet without evidence of vegetation. She was separated from ECMO support but developed acute renal failure requiring one week of hemodialysis. She was extubated on day ten of hospitalization and kidney function recovered completely. Neurologically she has remained without any deficits noted.

Bottom Line: This disease can cause significant morbidity and mortality if left untreated.She was treated with steroids with improvement of her symptoms and scheduled for close follow-up.In general patients with IHES that have cardiac involvement have poorer prognoses.

View Article: PubMed Central - PubMed

Affiliation: Florida Hospital for Children, Orlando, FL 32803, USA ; University of Central Florida, Orlando, FL 32827, USA.

ABSTRACT
Idiopathic Hypereosinophilic Syndrome (IHES) is a rare disease that can be difficult to diagnose as the differential is broad. This disease can cause significant morbidity and mortality if left untreated. Our patient is a 17-year-old adolescent female who presented with nonspecific symptoms of abdominal pain and malaise. She was incidentally found to have hypereosinophilia of 16,000 on complete blood count and nonspecific colitis and pulmonary edema on computed tomography. She went into cardiogenic shock due to papillary rupture of her mitral valve requiring extreme life support measures including intubation and extracorporal membrane oxygenation (ECMO) as well as mitral valve replacement. Pathology of the valve showed eosinophilic infiltration as the underlying etiology. The patient was diagnosed with IHES after the exclusion of infectious, rheumatologic, and oncologic causes. She was treated with steroids with improvement of her symptoms and scheduled for close follow-up. In general patients with IHES that have cardiac involvement have poorer prognoses.

No MeSH data available.


Related in: MedlinePlus