What is the best course of management for a patient with chest and abdominal pain, presenting with mild pulmonary edema, esophagitis, pulmonary arterial hypertension, hepatosplenomegaly, polycystic kidneys, and renal osteodystrophy, with a history of three-vessel coronary atherosclerosis and severe calcification of the abdominal aorta?

Management of Complex Multi-System Disease with Chest/Abdominal Pain

This patient requires immediate cardiology consultation and aggressive management of pulmonary arterial hypertension, esophagitis, and volume overload, with the primary focus on addressing the life-threatening PAH and preventing right heart failure progression.

Immediate Priorities

Address Pulmonary Arterial Hypertension (PAH)

• The pulmonary arterial trunk distension to 3.8 cm with evidence of pulmonary edema indicates significant PAH requiring urgent specialist evaluation 1
• PAH in the setting of polycystic kidney disease and renal osteodystrophy suggests this is secondary PAH (WHO Group 5 - unclear/multifactorial mechanisms), not isolated valvular disease 2, 1
• Cardiac catheterization is recommended for definitive hemodynamic assessment given the clinical symptoms and echocardiographic findings suggestive of pulmonary hypertension 1
• The presence of systemic venous congestion (hepatosplenomegaly, ascites) suggests significant right ventricular dysfunction, which is an uncommon finding in isolated pulmonary stenosis and indicates more severe disease 2

Manage Volume Overload

• Elevated right-sided heart pressures, edema, hepatosplenomegaly, and ascites should be treated with loop diuretics and aldosterone antagonists 2
• The mild pulmonary edema requires careful diuretic management to reduce preload without compromising cardiac output 2
• Monitor closely for worsening renal function given the polycystic kidney disease 2

Treat Esophagitis

• The distal esophageal wall thickening suggestive of esophagitis requires initiation of high-dose proton pump inhibitor therapy 3, 4
• Intravenous pantoprazole 40 mg daily can be used initially if oral intake is compromised, with demonstrated efficacy in achieving acid suppression within 45 minutes and maintaining control for 24 hours 3
• After initial control, transition to oral PPI therapy for 8 weeks, which has shown 86-87% healing rates for esophageal lesions 3

Secondary Evaluation Priorities

Cardiac Risk Assessment

• The three-vessel coronary atherosclerosis with severe aortic calcification requires stress imaging to screen for functional coronary artery disease, particularly given the chest pain presentation 2
• Markedly enlarged pulmonary artery aneurysms may rarely cause chest pain by compression of the left main coronary artery, which should be evaluated 2
• Serial troponin measurements are necessary given the chest pain presentation, even though the primary pathology appears non-ischemic 2

Investigate PAH Etiology

• Other causes of PAH must be systematically excluded, including peripheral pulmonary artery stenosis, chronic thromboembolic disease, and pulmonary veno-occlusive disease 2, 5
• The polycystic kidney disease and renal osteodystrophy suggest chronic kidney disease as a contributing factor to the PAH 2, 1
• High-resolution CT with angiographic acquisitions is useful for identifying underlying disorders and differentiating among various causes of secondary pulmonary hypertension 5

Ongoing Management Strategy

PAH-Specific Therapy

• Current treatment algorithms recommend combination therapy targeting the three specific pathways of endothelial dysfunction: endothelin, nitric oxide, and prostacyclin pathways 6
• The choice of dual versus triple therapy depends on initial severity assessment using multiparametric risk stratification 6
• The main treatment goal is to achieve low-risk status, with further escalation required if this is not achieved at follow-up 6

Monitoring and Follow-Up

• Patients with significant PAH should be followed with clinical examination, Doppler echocardiography, BNP, chest x-ray, 6-minute walk test, and cardiac catheterization for initial and follow-up evaluation 1
• Regular assessment of right ventricular function is critical, as progressive RV dilation suggests worsening disease or associated lesions 2
• The prominent lymph nodes in bilateral subpectoral and axillary regions (up to 8 mm) require follow-up imaging to exclude malignancy or systemic inflammatory conditions 2

Critical Pitfalls to Avoid

Do Not Delay Specialist Referral

• PAH with evidence of right heart failure requires immediate cardiology/pulmonary hypertension specialist consultation 1, 6
• Lung transplantation may be indicated in the most severe patients already on maximal medical therapy 6

Avoid Medications That Worsen Outcomes

• Beta-blockers and calcium channel blockers may be used for dynamic outflow tract obstruction similar to hypertrophic cardiomyopathy, but their use in established PAH with right heart failure requires specialist guidance 2
• Thiazide diuretics should be used cautiously given the renal disease and potential effects on glucose metabolism 2

Monitor for Complications

• Cyanosis development would suggest right-to-left shunting through a patent foramen ovale due to increased right atrial pressure, indicating severe decompensation 2
• The central venous catheter tip positioned in the right atrium requires repositioning to the superior vena cava to reduce risk of arrhythmias and thrombosis 2
• Severe aortic calcification ("porcelain aorta") significantly increases surgical risk if cardiac intervention becomes necessary 2

Address Renal Disease Impact

• Renal parenchymal disease is the most common cause of secondary hypertension and contributes to vascular calcification 2
• The manifestations of renal osteodystrophy indicate advanced chronic kidney disease requiring nephrology co-management 2
• Vascular calcification is common in CKD and predicts poor patient outcomes, with pelvic arterial calcification being an independent predictor of mortality 7