Pulmonary Thromboendarterectomy for Pulmonary Hypertension Before Considering Transplant.

BACKGROUND: In cases of chronic thromboembolic pulmonary hypertension (CTEPH), referral for possible surgical intervention is important because surgery can be curative. Surgery necessitates cardiopulmonary bypass and deep circulatory arrest with pulmonary thrombectomy and bilateral endarterectomy (PTE). If surgery fails, lung transplant is the next best surgical option. Medical treatment is also an important adjunct. CASE REPORT: A 35-year-old female presented 3 months after a pulmonary embolus was found to be completely occluding her left pulmonary artery. She was found to have pulmonary hypertension with a pulmonary artery pressure of 81/33 mmHg, with a mean pressure of 52 mmHg. The right atrial pressure was also severely elevated at 29 mmHg, and her echocardiogram revealed severe tricuspid regurgitation and severe right ventricular dysfunction. She underwent PTE and postoperatively was followed by the heart failure team. Her 6-minute walk distance improved from 396 meters at 1 month to 670 meters at 7 months, and her pulmonary artery pressure improved significantly to 55/17 mmHg with a mean pressure of 31 mmHg. The patient's right atrial pressure also improved significantly from 29 mmHg to 13 mmHg. CONCLUSION: CTEPH is likely underrecognized, and patients with pulmonary hypertension or a history of pulmonary embolism should be screened for CTEPH. This case illustrates the surgical treatment for CTEPH and discusses alternative and adjunctive treatments. Residual pulmonary hypertension after PTE occurs in approximately 35% of patients. Overall, 4-year mortality rates after surgery appear to be approximately 15%, and mortality rates correlate with the postoperative pulmonary vascular resistance. Recognition of chronic pulmonary thromboembolic disease as the etiology of pulmonary hypertension warrants evaluation for surgery.

Successful pulmonary artery embolectomy in a patient with a saddle Wilms tumor embolus.

Denys-Drash syndrome is a genetic disorder characterized by ambiguous genitalia, cryptorchidism, nephrotic syndrome, and a high predilection for Wilms tumor with intravascular invasion. We report a 5-year-old male with Denys-Drash syndrome who rapidly developed Wilms tumor with vascular invasion, subsequent saddle tumor embolus, and required emergent embolectomy. This case illustrates the rapid emergence of Wilms tumor in a patient with Denys-Drash syndrome and the importance of considering embolectomy over thrombolytic therapy for PE in this population, given a high likelihood of tumor embolus.

Closure of external tunnel Fontan fenestration: a novel use of the Amplatzer vascular plug.

BACKGROUND: Newer modifications of the Fontan operation include the external tunnel conduit with placement of an elongated type of fenestration. Atrial septal defect closure devices used traditionally to close fenestrations have short connecting waists with retention discs on each side. These may not be suitable for this type of Fontan fenestration. The length of the fenestration may not allow proper expansion of the retention discs on these devices. The Amplatzer vascular plug, a new occlusion device without a centering waist or retention discs designed to close vascular malformation, can be placed entirely within an elongated tube or vessel. METHODS: A retrospective review was used to study patients who underwent catheter closure of Fontan fenestration in our institution. Only patients whose procedure was performed after the commercial release of the Amplatzer vascular plug were included in the study. RESULTS: The vascular plug was implanted in 4 of 10 patients who underwent fenestration closure. The plug was successfully placed in all four patients without protrusion into either the systemic or pulmonary venous side of the baffle. There were no incidences of embolization, hemolysis, or infective endocarditis. All the patients experienced significant improvement in oxygen saturation. The technical aspects of this device are discussed as well as its safe and effective use. The satisfactory follow-up evaluation also is reported. CONCLUSIONS: This study outlines a novel use of the Amplatzer vascular plug for successful closure of selected Fontan fenestrations.

Congenital complete heart block: fetal management protocol, review of the literature, and report of the smallest successful pacemaker implantation.

Fetuses with complete heart block have an increased mortality with most deaths occurring in utero or during infancy. The cardiac evaluation of these fetuses is difficult since the ventricular rate is low and the heart is dilated. We have implemented a strategy that includes the biophysical profile, which assesses fetal well-being, in combination with the cardiovascular profile that assesses cardiac function and the circulation. We present two cases of fetal complete heart block in which early delivery was recommended due to worsening cardiovascular profile scores. Biophysical profile scores were normal. Both babies were successfully treated, despite having risk factors that predicted poor outcomes. We hypothesize that our management protocol initiated intervention before fetal compromise, hydrops, and myocardial damage occurred. We recommend an evaluation of heart function in addition to an assessment of fetal well-being in fetuses with complete heart block. Early delivery should be considered if there is evidence of distress and/or deteriorating cardiac function.